TITLE: Sudden Sensorineural Hearing Loss SOURCE: Grand Rounds Presentation, UTMB, Dept o!

Otolar"ngolog" D#TE: $une %&, '((% RESIDE)T PH*SICI#): C+ristop+er Muller, MD ,#CULT* PH*SICI#): $e!!re" -ra.e/, MD SERIES EDITOR: ,ran/is B 0uinn, $r , MD
"This material was prepared by resident physicians in partial fulfillment of educational requirements established for the Postgraduate Training Program of the UTMB Department of Otolaryngology/ ead and !ec" #urgery and was not intended for clinical use in its present form$ %t was prepared for the purpose of stimulating group discussion in a conference setting$ !o warranties& either e'press or implied& are made with respect to its accuracy& completeness& or timeliness$ The material does not necessarily reflect the current or past opinions of members of the UTMB faculty and should not be used for purposes of diagnosis or treatment without consulting appropriate literature sources and informed professional opinion$"

Introduction
Sudden +earing loss 1SHL2 is a 3edi/al e3ergen/" !or 4+i/+ de!initi5e diagnosis and treat3ent is still largel" un6no4n It 4as !irst des/ri.ed in t+e literature ." De 7le5n in %899 SHL generall" a re!ers to +earing loss o! sensorineural origin It +as .een de!ined !or resear/+ purposes and +as .een a//epted ." 3ost aut+orities as &( dB or 3ore sensorineural +earing loss o5er at least t+ree /ontiguous audio3etri/ !re:uen/ies o//urring 4it+in & da"s or less Esti3ates report appro;i3atel" %<,((( reported /ases o! SHL per "ear 4orld4ide 4it+ 9((( o! t+ose o//urring in t+e United States One in e5er" %(,((( to %<,((( people 4ill su!!er !ro3 t+is /ondition, 4it+ t+e +ig+est in/iden/e o//urs .et4een <( and =( "ears o! age T+e lo4est in/iden/e is .et4een '( and &( "ears o! age O! t+e patient su!!ering !ro3 SHL, '> are .ilateral In 3ost series, t+e in/iden/e 4as nearl" e:ual in 3en and 4o3en T+ere are 3an" potential /auses o! SHL, .ut despite e;tensi5e e5aluation, t+e 3a?orit" o! /ases elude de!initi5e diagnosis and t+ere!ore, re3ain idiopat+i/ Reports esti3ate t+at t+e etiolog" o! SHL is diagnosed in onl" %(> o! /ases Suggested /auses o! idiopat+i/ sudden sensorineural +earing loss 1ISS)HL2 in/lude 5iral in!e/tions, i33unologi/, 5as/ular /o3pro3ise, and intra/o/+lear 3e3.rane .rea6s It is unli6el" t+at an" single one o! t+ese pat+op+"siologi/ pro/esses e;plains all /ases o! ISS)HL Treat3ent regi3ens ai3ed at addressing t+e underl"ing pro.le3 in ea/+ o! t+ese states +a5e .een suggested in/luding de/reasing /o/+lear in!la33ation, i3pro5ing inner ear .lood !lo4 and o;"genation, and reesta.lis+ing t+e endo/o/+lear potential

Etiology
T+e etiolog" o! SHL /an .e .ro6en do4n into .road /ategories: 1%2 5iral and in!e/tious, 1'2 autoi33une, 1&2 la."rint+ine 3e3.rane rupture@trau3ati/, 192 5as/ular,

1<2 neurologi/, and 1=2 )eoplasti/ T+ere are 3ultiple /onditions 4it+in ea/+ o! t+ese /ategories t+at +a5e .een asso/iated 4it+ sudden +earing loss T+e !ollo4ing is a partial list o! reported /auses o! SHL: Infectious Meningo/o//al 3eningitis Herpes5irus 1si3ple;, Aoster, 5ari/ella, /"to3egalo5irus( Mu3ps Hu3an i33unode!i/ien/" 5irus Lassa !e5er M"/oplas3a Cr"pto/o//al 3eningitis To;oplas3osis S"p+ilis Ru.eola Ru.ella Hu3an spu3aretro5irus #utoi33une inner ear disease 1#IED2 Ul/erati5e /olitis Relapsing pol"/+ondritis Lupus er"t+e3atosus Pol"arteritis nodosa CoganBs s"ndro3e CegenerBs granulo3atosis Peril"3p+ !istula Inner ear de/o3pression si/6ness Te3poral .one !ra/ture Inner ear /on/ussion Otologi/ surger" 1stapede/to3"2 Surgi/al /o3pli/ation o! nonotologi/ surger" -as/ular disease@alteration o! 3i/ro/ir/ulation -as/ular disease asso/iated 4it+ 3ito/+ondriopat+" -erte.ro.asilar insu!!i/ien/" Red .lood /ell de!or3a.ilit" Si/6le /ell disease Cardiopul3onar" ."pass Multiple s/lerosis ,o/al pontine is/+e3ia Migraine #/ousti/ neuro3a

Autoimmune

Traumatic

Vascular

Neurologic

Neoplastic

Leu6e3ia M"elo3a Metastasis to internal auditor" /anal Meningeal /ar/ino3atosis Contralateral dea!ness a!ter a/ousti/ neuro3a surger"

History and Physical

E5aluation and 3anage3ent o! SHL s+ould .e /onsidered 3edi/all" urgent, i! not an e3ergen/" T+e pri3ar" goal is to rule out an" treata.le /auses Diagnosti/ e5aluation o! t+e patient 4it+ sudden +earing loss .egins 4it+ a t+oroug+ +istor" and p+"si/al e;a3 Details o! t+e /ir/u3stan/es surrounding t+e +earing loss and t+e ti3e /ourse o! its onset s+ould .e eli/ited #sso/iated s"3pto3s, su/+ as tinnitus, 5ertigo or diAAiness, and aural !ullness s+ould also .e as6ed a.out Clini/al e;perien/e +as s+o4n t+at a.out oneDt+ird o! patients note t+eir +earing loss upon !irst a4a6ening in t+e 3orning, and t+at a.out oneD +al! t+e /ases 4ill +a5e asso/iated 5ertigo Patients s+ould also .e :uestioned a.out pre5ious otologi/ surger", ototo;i/ drug use, and pre5ious or /on/urrent 5iral or upper respirator" tra/t in!e/tions #n" +istor" o! trau3a, straining, di5ing, !l"ing, and intense noise e;posure s+ould .e noted Past 3edi/al +istor" o! ot+er diseases asso/iated 4it+ sudden +earing loss s+ould also .e o.tained su/+ as dia.etes, autoi33une disorders, 3alignan/ies, neurologi/ /onditions 13ultiple s/lerosis2, and +"per/oagula.le states #!ri/anD#3eri/ans s+ould .e as6ed a.out si/6le /ell disease # /o3plete +ead and ne/6 e;a3 s+ould .e per!or3ed on all patients 4it+ sudden +earing loss More o!ten t+an not, t+e e;a3 4ill .e unre3ar6a.le, +o4e5er, an" pro/esses su/+ as 3iddle ear e!!usions, in!e/tions, /+olesteato3a, and /eru3en i3pa/tion s+ould .e e;/luded # t+oroug+ neurologi/al e;a3 in/luding Ce.er and Rinne, /ere.ellar and 5esti.ular testing s+ould .e per!or3ed

Diagnostic Testing
#n audiogra3 1pure tone, spee/+, t"3pano3etr", in/luding stapedial re!le; testing2 s+ould .e per!or3ed on all patients 4it+ sudden +earing loss T+e audiogra3 is t+e !oundation o! t+e diagnosis and pro5ides prognosti/ in!or3ation Serial testing pro5ides do/u3entation o! t+e progression or resolution o! t+e +earing loss and response to treat3ent In addition it 3a" +elp e;/lude patients 4it+ se/ondar" gain or 4it+ pseudo+"pa/usis T+e !ollo4ing is a list o! la.orator" studies t+at /an .e ordered Initial s/reening tests s+ould .e dire/ted .ased on +istor" and suspe/ted /onditions %2 '2 &2 92 <2 =2 Co3plete .lood /ount 1CBC2 Er"t+ro/"te sedi3entation rate 1ESR2 Glu/ose C+olesterol@trigl"/erides T&, T9, TSH PT, PTT

E2 -DRL, RT#D#BS 1MH#DTP2 F2 HI82 L"3e titer Magneti/ resonan/e i3aging 1MRI2 is re/o33ended ." t+e 3a?orit" o! aut+ors !or patients 4it+ as"33etri/ +earing loss In one sur5e" o! E8 otolar"ngologists, &F> 4ould order i3aging on t+e patientBs initial 5isit MRI is use!ul in e5aluating !or a/ousti/ tu3ors, 3ultiple s/lerosis and /ere.ro5as/ular a//idents T+ere are so3e proponents o! !ollo4ing t+ese patients and i3aging onl" i! as"33etri/ +earing persists Ho4e5er, Berg et al , in a series o! a/ousti/ neuro3as s+o4ed t+at %&> presented 4it+ sudden +earing loss, and o! t+ese '&> re/o5ered auditor" !un/tion

Known Treatable auses of !udden !ensorineural Hearing "oss

Autoimmune #utoi33une +earing loss 3a" .e asso/iated 4it+ or part o! s"ste3i/ autoi33une diseases su/+ as CoganGs s"ndro3e, CegenerBs granulo3atosis, pol"arteritis nodosa, te3poral arteritis, BuergerBs disease 1t+ro3.oangitis o.literans2, and s"ste3i/ lupus er"t+o3atosis, or 3a" .e pri3ar" to t+e inner ear T+e pat+ogenesis o! i33uneD 3ediated sensorineural dea!ness and 5esti.ular d"s!un/tion are un/lear, .ut are presu3ed to in/lude: 5as/ulitis o! 5essels suppl"ing t+e inner ear, autoanti.odies dire/ted against inner ear antigeni/ epitopes, or /rossDrea/ting anti.odies #utoi33une +earing loss i3plies t+at inner ear proteins are re/ogniAed i33unologi/all" as !oreign or nonDsel! So3e aut+ors /ontend t+at t+ere is no su/+ entit" as autoi33une +earing loss as t+ese putati5e inner ear antigens are as o! "et un6no4n CoganGs s"ndro3e 1CS2 is an autoi33une disease o! t+e /ornea and 5esti.uloauditor" apparatus t+at 4as !irst des/ri.ed ." Cogan, an op+t+al3ologist, in t+e %89(s It o//urs pri3aril" in "oung adults 1a5erage age o! onset ''D'8 "ears2 and t"pi/all" presents 4it+ interstitial 6eratitis 1I72 and MeniereGsDli6e atta/6s o! 5ertigo, ata;ia, tinnitus, nausea, 5o3iting, and +earing loss 4+i/+ de5elop 4it+in se5eral 3ont+s o! ea/+ ot+er CS 3a" also .e asso/iated 4it+ ot+er s"ste3i/ 3ani!estations o! t+e in!la33ator" pro/ess su/+ as Ta6a"asuGsDli6e or 3ediu3DsiAed 5essel 5as/ulitis #ppro;i3atel" %(> o! patients de5elop aortitis 4it+in 4ee6s to "ears a!ter t+e onset Hearing !lu/tuation in CS /oin/ides 4it+ disease e;a/er.ations and re3issions Its /ourse o!ten /ul3inates in dea!ness One series reported t+at %' out o! %F patients 1=E>2 de5eloped .ilateral dea!ness T+e /ause o! CS is un6no4n Mi/ro.ial etiolog" +as .een suggested ." so3e as URTI pre/ede nearl" 9(> o! patients 4+o present 4it+ t+is disease Clini/al parallels .et4een s"p+ilis and CS +a5e led so3e to .elie! t+at CS 3a" .e /aused ." Borrelia .urgdor!eri Ho4e5er, e5iden/e so !ar +as .een in/on/lusi5e T+ere +a5e also .een lin6s to C+la3"dia spe/ies 4it+ CS and is an area o! ongoing in5estigation Te3poral .one +istopat+ologi/ studies done at autops" o! patients 4it+ CS are /+ara/teriAed ." /+roni/ in!la33ation in/luding: in!iltration o! t+e spiral liga3ent 4it+ l"3p+o/"tes and plas3a /ells, endol"3p+ati/ +"drops, degenerati5e /+anges in t+e organ o! Corti, and

de3"elination and atrop+" o! t+e 5esti.ular and /o/+lear .ran/+es o! t+e eig+t /ranial ner5e T+ere is no /riteria /urrentl" esta.lis+ed !or t+e diagnosis o! CS T+e general t+in6ing is t+at t+e diagnosis re:uires /lini/al signs o! .ot+ e"e and inner ear in!la33ation Cor6Dup s+ould in/lude an audiogra3 and la.orator" tests in/luding CBC, ESR, and RPR I3aging in/luding MRI and@or CT s+ould .e done pri3aril" to rule out /ere.ropontine angle tu3ors and ot+er disorders MRI 3a" s+o4 en+an/e3ent o! 5esti.ular and /o/+lear stru/tures 4it+ gadoliniu3 T+e /ornerstone o! t+erap" is /orti/osteroids: topi/al !or I7 and oral !or 5esti.uloauditor" in5ol5e3ent Most aut+ors suggest using prednisone %3g@6g !or 'D9 4ee6s 4it+ a su.se:uent rapid taper !or /ases o! /o3plete resolution and slo4 taper !or t+ose 4it+ in/o3plete response T+e .est out/o3e is in patients in 4+o3 t+erap" .egins s+ortl" a!ter t+e onset o! s"3pto3s ,or patients 4+o +a5e !ailed steroid treat3ent and de5eloped .ilateral dea!ness, /o/+lear i3plantation s+ould .e /onsidered and +as .een appro5ed ." t+e ,D# in t+ese patients Traumatic Trau3ati/ .rea6s in t+e 3e3.ranous la."rint+ are a//epted /auses o! sudden +earing loss Co/+lear 3e3.rane .rea6s 3a" .e eit+er intra/o/+lear, as is t+oug+t to o//ur in MeniereBs disease, or in5ol5e t+e la."rint+ine o5al and@or round 4indo4s 4it+ a resultant peril"3p+ !istula T+e patients +istor" 4ill usuall" eli/it an in/iting e5ent su/+ as a .lo4 to t+e +ead, sneeAing, .ending o5er, li!ting a +ea5" o.?e/t, e;posure to sudden /+anges in .aro3etri/ pressure 1su/+ as during !l"ing or di5ing2, or e;posure to a loud noise Patients 4+o are t+eoreti/all" at +ig+ ris6 !or !istuliAation are t+ose 4+o +a5e undergone anato3i/al alterations su/+ as stapede/to3" patients and in /+ildren 4it+ inner ear ano3alies, su/+ as t+e Mondini 3al!or3ation and large 5esti.ular a:uedu/ts 4+ere in/reases in CS, pressure 3a" .e trans3itted to t+e inner ear 3ore easil" In /lini/al pra/ti/e, +o4e5er, patients 4+o +a5e undergone stapede/to3" 4+o return to t+eir nor3al li!est"les in/luding s/u.a di5ing and !l"ing +a5e not resulted in an o.5ious in/rease in de5elop3ent o! s"3pto3s o! peril"3p+ati/ !istulas Currentl" t+ere is no de!initi5e test to diagnose peril"3p+ !istulae ot+er t+an intraoperati5e o.ser5ation o! lea6age o! peril"3p+ Diagnosis is 3ade ." t+e /lini/al +istor" o! sudden or rapidl" progressi5e +earing loss a!ter one o! t+e a.o5e listed in/iting e5ents In!la33ation, granulo3a, or neoplasia disorders t+at /an 3i3i/ a peril"3p+ !istula s+ould .e ruled out 1i e 4it+ MRI, MH#TP, and ESR2 7o+ut proposes t+at t4o o! t+e !ollo4ing 5esti.ular s"3pto3s are re:uired !or diagnosis: /onstant d"se:uili.riu3, a positi5e !istula test 1Henne.ertBs sign2, and a positional n"stag3us Testing !or TullioBs p+eno3enon s+ould also .e done Initial treat3ent s+ould in/lude < da"s o! stri/t .ed rest 4it+ t+e +ead o! .ed ele5ated t+irt" degrees T+e patient s+ould a5oid straining or +ard nose .lo4ing Stool so!teners 3a" .e gi5en So3e suggest dail" audiogra3s I! t+e patient +as i3pro5e3ent, = 3ore 4ee6s o! 3odi!ied p+"si/al a/ti5it" s+ould .e !ollo4ed I! no i3pro5e3ent is seen a!ter !i5e da"s, surgi/al t+erap" in/luding 3iddle ear e;ploration 4it+ pat/+ing o! t+e peril"3p+ati/ !istula s+ould .e per!or3ed Neoplastic

#/ousti/ neuro3as are usuall" asso/iated 4it+ graduall" progressi5e +earing loss Ho4e5er, t+e in/reasingl" 4idespread use o! CT and MRI i3aging o! patients +as indi/ated t+at nearl" %(> to %8> o! patients 4it+ a/ousti/ tu3ors 3a" present 4it+ SHL T+e rationale !or i3aging patients 4it+ unilateral +earing loss, a//epted ." 3ost aut+orities is t+at, alt+oug+ un/o33on, t+eir s"3pto3s 3a" .e due to neoplas3 It +as .een esti3ated t+at %> o! patients 4it+ as"33etri/ SHL 4ill +a5e an a/ousti/ tu3or In addition, t+ese i3aging studies /an pi/6 ot+er neurologi/ disorders su/+ as 3ultiple s/lerosis and is/+e3i/ /+anges 4+i/+ 3a" .e asso/iated 4it+ SHL

Idiopathic !udden !ensorineural Hearing loss #I!!NH"$% Etiologic Theories

Viral #lt+oug+ de!initi5e proo! re3ains to .e esta.lis+ed, t+e /urrent .elie! is t+at 5iral /o/+leitis is t+e /ulprit in t+e 3a?orit" o! /ases o! ISS)HL In %8F&, serologi/ studies per!or3ed ." Cilson and /olleagues de3onstrated a statisti/all" signi!i/ant in/rease in 5iral sero/on5ersion in patients 4it+ ISS)HL /o3pared 4it+ /ontrols !or CM- as 4ell as in!luenAa B, 3u3ps, ru.eola, and 5ari/ella Aoster 5iruses T+e" loo6ed at %'' patients o5er a & "ear period 4it+ SHL and !ound t+at =&> +ad do/u3ented 5iral titer sero/on5ersion, /o3pared 4it+ 9(> o! /ontrols -eltri and /oD4or6ers de3onstrated sero/on5ersion at a rate o! =<> in EE un3at/+ed patients 4it+ SHL Te3poral .one +istopat+ologi/ studies +a5e de3onstrated /+anges t+at are /onsistent 4it+ 5iral in!e/tion S/+u6ne/+t and Dono5an loo6ed at t+e +istopat+olog" o! t4el5e te3poral .ones !ro3 patients 4it+ ISS)HL and re5ie4ed ten ot+er /ases !ro3 t+e literature T+e" /o3pared t+ese 4it+ /ases !ro3 patients 4it+ 6no4n 5iral la."rint+itis and !ound si3ilar +istopat+ologi/ !indings in/luding atrop+" o! t+e organ o! Corti, te/torial 3e3.rane, stria 5as/ularis, /o/+lear ner5e, and 5esti.ular organ T+is /ontrasts to t+e !indings in ani3al 3odels o! +earing loss o! 5as/ular origin, 4+i/+ /auses e;tensi5e /o/+lear !i.rosis and ossi!i/ation In %888, #l.ers and S/+ir3 dire/tl" introdu/ed HS-D% into t+e round 4indo4 o! guinea pigs Histopat+ologi/ e;a3ination o! t+e /o/+lea re5ealed si3ilar !indings as t+ose !ound ." S/+u6ne/+t and Dono5an T+e e;a/t 3e/+anis3 ." 4+i/+ 5iruses /ause +earing loss +as not .een de!initi5el" pro5en Ho4e5er, studies suggest t+at t+ere is a signi!i/ant /ontri.ution o! t+e +ost i33une response to t+e pat+ologi/ /+anges in t+e 3e3.ranous la."rint+ and su.se:uent +earing loss o! patients 4it+ ISS)HL Harris et al s+o4ed t+at in e;peri3ental guinea pigs 4it+ CM-Dindu/ed SS)HL, i33unosuppressed ani3als s+ared a lesser degree o! S)HL /o3pared 4it+ i33uno/o3petent /ontrols In addition, se5eral studies in +u3ans +a5e s+o4n .ene!i/ial e!!e/ts o! steroids on re/o5er" !ro3 ISS)HL Sin/e t+e pri3ar" e!!e/t o! steroids is antiDin!la33ator", t+is lends additional support to +ost i33une 3ediated /o/+leitis in response to a 5irus Dire/t identi!i/ation o! 5iruses or 5iral antigens !ro3 t+e peril"3p+ o! a a!!e/ted patients pro5ides so3e o! t+e strongest e5iden/e !or t+e in5ol5e3ent o! 5iruses in +earing loss #lso, ani3al studies t+at de3onstrate t+e a.ilit" o! 5iruses to in!e/t t+e inner ear lend !urt+er support Da5is and $o+nson de3onstrated t+e a.ilit" o! ru.eola and 3u3ps

to in!e/t t+e inner ears o! ani3al 3odels using i33uno!luores/ent antigen studies Cest3ore et$ al su.se:uentl" /ultured t+e 3u3ps 5irus !ro3 t+e peril"3p+ o! a patient su!!ering !ro3 SHL and in %8E8, Da5is and /oD4or6ers /ultured CM- !ro3 t+e peril"3p+ o! in!ant 4it+ /ongenital CM- in!e/tion In %8F%, !urt+er studies using i33uno!luores/en/e s+o4ed CM- antigens on t+e stria 5as/ularis, dar6 /ells and ReissnerBs 3e3.rane o! an in!ant 4+o died o! a CM- in!e/tion Autoimmune M/Ca.e !irst des/ri.ed autoi33une inner ear disease 1#IED2 in %8E8 He reported on eig+teen patients 4it+ .ilateral rapidl" progressi5e sensorineural +earing loss 1S)HL2 !or 4+i/+ no identi!ia.le /ause /ould .e !ound E5iden/e o! autoi33unit" in t+ese patients in/luded a positi5e l"3p+o/"te in+i.ition test In t+is test, patientBs seru3 4as /o3.ined 4it+ antigens deri5ed !ro3 t+e 3e3.ranous la."rint+ o! patients 4+o +ad undergone transla."rint+ine rese/tion o! a/ousti/ tu3ors #ll patientBs sera rea/ted to t+e antigens 4+ile /ontrols did not #dditional e5iden/e o! autoi33une etiolog" is indi/ated ." t+e !a/t t+at patients +ad su.stantial +earing i3pro5e3ent 4it+ steroid treat3ent ,urt+er3ore, +istopat+ologi/ e;a3ination o! one patientBs te3poral .one re5ealed 5as/ulitis M/Ca.e proposed t+at t+e diagnosis o! #IED .e .ased on positi5e i33une la.orator" tests and .ene!i/ial treat3ent response T+e /lini/al pi/ture o! #IED usuall" /onsists o! rapidl" progressi5e .ilateral sensorineural +earing loss usuall" in t+e a.sen/e o! ot+er s"ste3i/ 3ani!estations distinguis+ing it !ro3 ot+er 6no4n autoi33une disorders dis/ussed pre5iousl" #ppro;i3atel" <(> o! patients 4ill /o3plain o! diAAiness Episodi/ lig+tD +eadedness or 3ild ata;ia is 3ore /o33on t+an true 5ertigo #lso, t+ese episodes o//ur 3ultiple ti3es dail" during a/ti5e disease as opposed to t4o or t+ree dis/rete episodes per 4ee6, as is seen in MeniereBs disease O//asionall" s"3pto3s o! pressure and tinnitus /an o//ur T+e s"3pto3s o!ten progress o5er 4ee6s or 3ont+s .ut /an also present as sudden +earing loss or protra/ted disease o5er 3an" "ears Most patients present 4it+ .ilateral disease, and 4+en diAAiness is present, 5esti.ular testing usuall" re5eals .ilateral redu/ed response #IED +as a slig+t predo3inan/e in 3iddleDaged !e3ales, .ut /an o//ur in .ot+ se;es and /an .egin in /+ild+ood T+e diagnosis o! #IED is .ased !or t+e 3ost part on t+e presen/e o! .ilateral progressi5e sensorineural +earing loss and response to t+erap" Hug+es proposes t+at t+e t4o 3ost /lini/all" +elp!ul tests !or diagnosing #IED are t+e l"3p+o/"te trans!or3ation test 1LTT2 and t+e Cestern .lot i33unoassa", +o4e5er, t+ese are rarel" used ." 3ost pra/titioners .e/ause o! a5aila.ilit" T+e sensiti5it" and spe/i!i/it" !or LTT are esti3ated to .e <(DF(> and 8&> respe/ti5el" 4it+ positi5e predi/ti5e 5alues ranging !ro3 <=DE&> depending on t+e disease pre5alen/e in t+e tested population C+en applied to +ig+ ris6 populations 1patients 4it+ .ilateral rapidl"Dprogressi5e S)HL2, t+e Cestern .lot +as a sensiti5it" o! FF>, a spe/i!i/it" o! F(>, and an o5erall positi5e predi/ti5e 5alue o! 8'> T+e natural +istor" o! #IED is not 6no4n, +o4e5er, /lini/al e;perien/e re5eals t+at t+e disease 4a;es and 4anes Testing o! patients s+ould, t+ere!ore, .e per!or3ed during periods o! disease a/ti5it" and .e!ore treat3ent is initiated

Currentl", tests !or #IED are not routinel" used e;/ept at /ertain /enters 1Cle5eland Clini/ and t+e Massa/+usetts E"e and Ear In!ir3ar"2 and in e;peri3ental trials T+e 3a?or dra4.a/6 to t+ese studies is t+e la/6 o! a5aila.ilit" I! testing is desired, sa3ples o! 4+ole .lood !ro3 patients /an .e 3ailed to t+e Cle5eland Clini/ ,oundation Regional La.orator" ." o5ernig+t /arrier !or LTT T+e test /osts H%'( (( and results ta6e appro;i3atel" se5en da"s T+e .est t+eoreti/al test !or #IED 4ould .e a test !or 3ar6er spe/i!i/ !or #IED #tte3pts +a5e .een 3ade in t+is area and are pro3ising In %88(, Harris and /olleagues pu.lis+ed t+e results o! studies 4+i/+ dis/o5ered, using Cestern .lot, an antiD=F6d autoanti.od" in t+e sera o! patients 4it+ rapidl" progressi5e S)HL Sin/e t+en, ot+er studies +a5e /on!ir3ed t+ese !indings O5erall ''> to <F> o! sera o! patients 4it+ rapidl" progressi5e S)HL 4ill /ontain t+is anti.od" Harris +as su.se:uentl" reported a 89> spe/i!i/it" !or test /orrelating results 4it+ responsi5eness to t+erap" and disease a/ti5it" Studies ." Billings and Harris are no4 sear/+ing !or t+e spe/i!i/ antigen in5ol5ed in #IED So !ar t+e" +a5e isolated a =F6d protein t+at is u.i:uitous in t+e inner as 4ell as ot+er areas o! t+e .od", and +a5e re/entl" reported e5iden/e t+at lin6s t+e =F6d antigen 4it+ +eat s+o/6 protein E( 1+sp E(2, a +ig+l" indu/i.le stress protein ,urt+er resear/+ is needed in t+is are to deter3ine t+e e;a/t relations+ip o! +sp E( to #IED and 4+et+er it pla"s an i3portant etiologi/ role or 4+et+er it is ?ust a .iDprodu/t o! t+e disease itsel! T+eories proposed are t+at 1%2 +u3an +sp E( 3a" +a5e a si3ilar a3ino a/id se:uen/e to an in!e/ting agent resulting in /rossDrea/ti5it" or 1'2 t+at t+ere 3a" .e a +sp E( spe/i!i/ to t+e inner ear t+at is seen as !oreign 4+en it is o5erDe;pressed during ti3es o! /+roni/ in!la33ation !ro3 an outside agent Treat3ent !or #IED is /ontro5ersial and 4idel" 5aried !ro3 pra/titioner to pra/titioner T+is is largel" due to t+e la/6 o! dou.leD.lind, prospe/ti5e /lini/al trials on t+e 3atter T+e general /onsensus is t+at steroids are e!!e/ti5e and s+ould .e used Most sour/es re/o33end prednisone %3g@6g@da" !or 9 4ee6s !ollo4ed ." a slo4 taper i! t+e patient responds I! t+e patient relapses on t+e taper, Harris re/o33ends instituting +ig+ dose prednisone and i! /ontinued re/urren/e o//urs 4it+ tapering, a /"toto;i/ agent su/+ as 3et+otre;ate 1MTI2 at a dosage o! E <D%< 3g 4ee6l" 4it+ !oli/ a/id, or /"/lop+osp+a3ide 1C"to;an2 s+ould .e instituted I! MTI is used, t+e steroids s+ould .e /ontinued a!ter starting t+e MTI as it ta6es one to t4o 3ont+s !or t+e prednisone sparing a!!e/ts o! MTI to .egin Most p+"si/ians .egin 4it+ MTI as it +as !e4er sideDe!!e/ts t+an C"to;an I! .ot+ prednisone and MTI are ine!!e/ti5e, /"to;an s+ould .e used It is i3portant to 3onitor !or side e!!e/ts o! .ot+ MTI and C"to;an 4it+ routine 3onitoring o! /o3plete .lood /ounts, platelets, L,Ts, U#, and ele/trol"tes T+ose on C"to;an s+ould 6eep 4ellD+"drated to pre5ent +e3orr+agi/ /"stitis Ot+er aut+ors su/+ as M/Ca.e are 3ore in !a5or o! starting /"toto;i/ drugs at t+e onset o! t+e illness He .elie5es t+at C"to;an is t+e pre!erred treat3ent o! #IED rat+er t+an steroids .e/ause t+ere is a +ig+er response rate to t+is drug Be/ause t+e diagnosis o! #IED is .ased partiall" on response to t+erap", !e4er patients 4it+ t+is diagnosis 4ould .e 3issed Vascular

It is not surprising t+at t+e /o/+leo5esti.ular .lood suppl" 3a" .e a!!e/ted ." /ir/ulator" disorders su/+ as e3.oli/ p+eno3enon, t+ro3.osis, 5asospas3, and +"per/oagula.le or +ig+ 5is/osit" states resulting in SS)HL T+e underl"ing pat+op+"siolog" /an .e e;plained ." t+e o//urren/e o! sudden ano;i/ in?ur" to t+e /o/+lea T+e /o/+lea is e;traordinaril" intolerant o! .lood suppl" disruptions Earl" studies ." 7i3ura and Perl3an in t+e%8<E re5ealed t+at 5as/ular o//lusion o! t+e la."rint+ine arter" in guinea pigs !or greater t+an t+irt" 3inutes led to irre5ersi.le loss o! /o/+lear !un/tion Suga and /oD4or6ers per!or3ed e;peri3ental e3.oliAations o! /o/+lear 5essels and s+o4ed loss o! /o/+lear a/tion potentials 4it+in =( se/onds Mu/+ o! t+e +ard e5iden/e !or a 5as/ular etiolog" o! SHL /o3es !ro3 +istopat+ologi/ /o3parison o! a !e4 +u3an te3poral .ones 4it+ t+ose o! ani3al 3odels Belal, in %8F(, loo6ed at t4o te3poral .ones !ro3 patients su!!ering !ro3 SHL He !ound si3ilar +istologi/ !indings to t+ose o! ani3al 3odels o! 5as/ular o//lusion to t+e /o/+leo5esti.ular apparatus in/luding e;tensi5e !i.rosis and ne4 .one !or3ation T+e .lood suppl" o! t+e 3e3.ranous la."rint+ is predo3inantl" deri5ed !ro3 +e la."rint+ine 1internal arter"2 4+i/+ is .ran/+ o! t+e anterior in!erior /ere.ellar arter" It 3a" less /o33onl" .e a dire/t .ran/+ !ro3 t+e .asilar arter" T+e la."rint+ine arter" enters t+e internal auditor" /anal and su.se:uentl" di5ides into t+e /o33on /o/+lear arter" and t+e anterior 5esti.ular arter" T+e /o33on /o/+lear arter" di5ides to !or3 t+e 3ain /o/+lear and 5esti.ulo/o/+lear .ran/+es Di5ision o! t+e 5esti.ulo/o/+lear arter" results in t+e posterior 5esti.ular arter" and t+e /o/+lear ra3us T+e arterial suppl" to t+e /o/+lea is su/+ t+at t+e .asal turn is !ed !irst ." t+e 3ain /o/+lear arter" 4it+ t+e /o/+lear ape; !ed last Based on t+is anato3" one 4ould e;pe/t o//lusion o! t+e la."rint+ine arter" to /ause .ot+ 5esti.ular and auditor" s"3pto3s 4+i/+ is supported ." +istopat+ologi/ !indings as des/ri.e a.o5e In addition, one 4ould e;pe/t te3porar" o//lusion in .lood !lo4 to a!!e/t lo4 !re:uen/" areas o! t+e /o/+lea !irst as t+ese areas are t+e 3ost distal in ter3s o! .lood suppl" SHL /an de5elop as a result o! a nu3.er a.nor3al /ir/ulator" states Patients 4it+ diseases su/+ a si/6le /ell ane3ia and Caldenstro3Gs 3a/roglo.uline3ia +a5e .een s+o4n to +a5e a +ig+er ris6 o! de5eloping SHL t+an t+e nor3al population Hearing loss in t+ese indi5iduals is usuall" re5ersi.le 4it+ treat3ent: O;"gen !or si/6le /ell disease and plas3ap+eresis !or Caldenstro3Gs Stro6es in5ol5ing t+e anterior in!erior /ere.ellar arter" are asso/iated 4it+ auditor" and 5esti.ular s"3pto3s .ut o!ten also a!!e/t /ere.ellar !un/tion SHL !ollo4ing /ardiopul3onar" ."pass +as also .een reported, 3ost li6el" resulting !ro3 e3.oli/ p+eno3enon Treatment T+e 3a?orit" o! /ases o! SHL treated ." otolar"ngologist are !or t+ose 4it+ no de!ina.le /ause T+e treat3ents !or /ases 4it+ 6no4n etiologies in5ol5e addressing t+e underl"ing /ondition 1i e treat3ent o!: a/ousti/ neuro3a 4it+ e;/ision, ototo;i/it" 4it+ /essation o! ototo;i/ drugs, 3ultiple s/lerosis 4it+ 3edi/al t+erap", e3.oli/ disease 4it+ anti/oagulants, si/6leD/ell /risis 4it+ o;"gen, .a/terial 3eningitis 4it+ anti.ioti/s and so on2 T+is dis/ussion 4ill .e li3ited to t+e treat3ents 4+i/+ +a5e .een proposed !or patients 4it+ no underl"ing /ause !or t+eir +earing loss .ased on t+e pre5ious 3entioned t+eories addressing t+e etiolog" o! ISS)HL

T+erap" !or ISS)HL is a su.?e/t o! /ontro5ers" T+e +ig+ spontaneous re/o5er" rate o! ISS)HL 19E> to =&>2 and its lo4 in/iden/e 3a6e 5alidation o! e3piri/al treat3ent 3odalities di!!i/ult Man" treat3ent regi3ens +a5e .een proposed !or ISS)HL Belo4 is a list o! treat3ent 3odalities 4+i/+ +a5e .een used and so3e o! 4+i/+ are /urrentl" used toda" !or t+e treat3ent o! ISS)HL: #ntiin!la33ator"@i33unologi/ agents Steroids Prostaglandin C"/lop+osp+a3ide Met+otre;ate H"do/+lorot+iaAide@tria3terene ,urose3ide #/"/lo5ir -ala/"/lo5ir <> /ar.on dio;ide 4it+ 8<> o;"gen 1Car.ogen2 Papa5erine Bup+enine 1n"lidrin2 )a!tidro!ur"l 1na!ron"l2 T+"3o;a3ine Prosta/"/lin )i/otini/ a/id Pento;i!"lline H"dro;"et+"l star/+ Lo4D3ole/ularD4eig+t de;tran Batro;o.in )i!edipine #3idotriAoate #/upunture Iron -ita3ins Pro/aine

Diureti/s #nti5iral agents -asodilators

-olu3e e;panders@+e3odilutors De!i.rinogenators Cal/iu3 antagonists Ot+er agents and pro/edures

# re5ie4 o! t+e literature /on!ir3s t+at t+e le5el o! e5iden/e !or treating t+is /ondition is li3ited and /onse:uentl" t+ere is a 4ide disparit" in /onsultantBs pra/ti/es In %8FE, Cil6ins and asso/iates treated %(8 4it+ a Js+otgunK regi3en t+at in/luded de;tran, +ista3ine, H"pa:ue, diureti/s, steroids, 5asodilators, and /ar.ogen in+alation T+e" !ound no statisti/all" signi!i/ant di!!eren/e in out/o3e .et4een patients treated and t+ose 4+o 4ere not treated In t+is stud" t+ere 4as no /ontrol group and treat3ent 4as de!ined as re/ei5ing t+e drug !or at least t+ree da"s Prospe/ti5e,

rando3iAed, dou.leD.lind studies +a5e .een done loo6ing at t+e de;tran 9(, pento;i!"lline, lo4D3ole/ularD4eig+t de;tran, and intra5enous pro/aine )one o! t+ese +a5e s+o4n signi!i/ant di!!eren/es in re/o5er" /o3pared to pla/e.o Despite t+e disappointing results in earl" trials o! steroid t+erap" in 4+i/+ lo4 doses 4ere used !or s+ort duration, later studies using +ig+er doses !or longer periods o! ti3e +a5e .een 3ore pro3ising in t+e treat3ent o! ISS)HL In %8F(, Cilson and /olleagues per!or3ed dou.leD.lind studies !or t+e treat3ent o! ISS)HL 4it+ oral steroids T+eir treat3ent /onsisted o! a De/adron taper o5er %(D%' da"s T+e" strati!ied t+eir patients .ased on audiogra3 and !ound t+at steroids +ad a signi!i/ant e!!e/t on t+e re/o5er" o! +earing in patients 4it+ +earing loss .et4een 9( and 8( d. Mos6o4itA et al /on!ir3ed CilsonBs !indings in %8F9 He de3onstrated an o5erall F8> re/o5er" rate !or t+ose treated 4it+ a t4el5e da" De/adron taper /o3pared 4it+ 99> re/o5er" !or t+ose not treated 4it+ steroids #gain, t+ese results 4ere !ound in a patients 4it+ audiogra3s in t+e so /alled Lsteroid e!!e/ti5e AoneL In .ot+ o! t+ese studies, t+e" !ound t+at patients 4it+ pro!ound +earing loss did not .ene!it signi!i/antl" !ro3 steroid use T+e" /on/luded t+at steroid appear to .e o! .ene!it onl" i! t+e in?ur" is partial and re5ersi.le In a re/ent re5ie4 ." Hug+es, +e re/o33ended treating 4it+ prednisone %3g@6g@da" !or at least ten da"s and up to one 3ont+ Hug+es also ad5o/ates treat3ent 4it+ diureti/s and lo4 salt diet #t+oug+ t+ere is no data to support t+is treat3ent, +is rationale is .ased on +istopat+ologi/ studies o! te3poral .ones in patients 4it+ autoi33une SHL 13ostl" CoganGs s"ndro3e2 4+i/+ +a5e s+o4n endol"3p+ati/ +"drops He pres/ri.es Ma;ide 1H"dro/+lort+iaAide '<3g and tria3terene2 on/e a da" Car.ogen, 4+i/+ is a /o3.ination o! 8<> o;"gen and <> /ar.on dio;ide +as .een used, and is still used ." so3e p+"si/ians in patients in 4+o3 a 5as/ular etiolog" is suspe/ted !or t+eir +earing loss Studies +a5e s+o4n t+at /ar.ogen in/reases t+e partial pressure o! o;"gen in peril"3p+ In addition /ar.on dio;ide is a 6no4n potent 5asodilator o! t+e 5esti.ulo/o/+lear 5as/ulature, resulting in in/reased .lood !lo4 #d3inistration o! Car.ogen 3ust .e done inD+ospital o5er t+ree da"s to 3onitor .lood pressure 4+i/+ 3a" in/rease Insuran/e /o3panies /urrentl" do not /o5er t+is treat3ent .e/ause it is /onsidered e;peri3ental )o /on/lusi5e data +as s+o4n Car.ogen in+alation to .e o! an" .ene!it o5er spontaneous re/o5er" ,inall", anti5irals +a5e re/entl" /o3e into !a5or in t+e treat3ent o! ISS)HL #ni3al 3odels o! 5iral la."rint+itis treated 4it+ prednisone and a/"/lo5ir /o3.ined +a5e s+o4n signi!i/antl" +ig+er rates o! +earing re/o5er" /o3pared to eit+er drug alone T+is /o3.ination t+erap" +as alread" pro5en its e!!e/ti5eness in Ra3sa" Hunt s"ndro3e and +erpes Aoster oti/us and +as also .een proposed !or t+e treat3ent o! BellGs pals" Studies are no4 ongoing to loo6 at t+e e!!i/a/" o! a/"/lo5ir in t+e treat3ent o! patients 4it+ ISS)HL In general, 3an" aut+orities treat patients e3piri/all" 4it+ a/"/lo5ir usuall" !or ten da"s Re/entl", a sur5e" 4as sent to %(( /onsultant otolar"ngologists in t+e United 7ingdo3 in:uiring a.out t+eir assess3ent and 3anage3ent o! patients presenting 4it+ SHL Results s+o4ed t+at EF> 4ould per!or3 routine .lood tests in/luding CBC, ESR, and s"p+ilis serolog" &F> 4ould order an MRI at t+e !irst presentation 8F <> o! t+e /onsultants 4ould treat 4it+ steroids alone or as part o! a /o3.ination 9%> treat 4it+ Car.ogen, &%> 4it+ a/"/lo5ir and &<> 4it+ .eta+istine

Prognosis
Pu.lis+ed series report spontaneous re/o5er" rates !or patients 4it+ SHL range !ro3 9E> to =&> T+ese re5ie4s /o3.ined patients 4it+ partial and /o3plete re/o5er" and patients 4ill all audiogra3 t"pes ,our 5aria.les +a5e .een s+o4n to a!!e/t re/o5er" !ro3 ISS)HL: 1%2 ti3e sin/e onset, 1'2 audiogra3 t"pe, 1&2 5ertigo, and 192 age In %8F9, B"l pu.lis+ed a prospe/ti5e stud" /ondu/ted o5er F "ears t+at e5aluated ''< patients 4it+ SHL ,a/tors e5aluated in/luded age, tinnitus, 5ertigo, audiogra3 pattern, ti3e elapsed !ro3 onset o! +earing loss to initial 5isit, and ESR le5el 4it+ respe/t to re/o5er" His !indings 4ere as !ollo4s: %2 Time since onset D His stud" /on!ir3ed t+at t+e sooner t+e patient 4as seen and t+erap" initiated, t+e .etter t+e re/o5er" <=> o! patients presenting 4it+in t+e !irst se5en da"s o! t+eir +earing loss re/o5ered /o3pared to 'E> 4+o presented t+irt" da"s or later He noted t+at t+ere is so3e sel!Dsele/tion .ias 4+ere." t+ose t+at re/o5er rapidl" do not see6 3edi/al aid '2 Age D T+e a5erage age !or t+ose re/o5ering totall" 4as 9% F "ears T+ose under %<"ears and o5er =( "ears +ad signi!i/antl" poorer re/o5er" rates &2 Vertigo D Patients 4it+ se5ere 5ertigo +ad signi!i/antl" 4orse out/o3es t+an patients 4it+ no s"3pto3s o! 5ertigo '8> o! patients 4it+ 5ertigo re/o5ered /o3pared to <<> 4it+ no 5ertigo 92 Audiogram D Patients 4it+ pro!ound +earing loss signi!i/antl" de/reased re/o5er" rates /o3pared to all ot+er groups 1''> 4it+ /o3plete re/o5er"2 Ot+er series +a5e s+o4n t+at patients 4it+ 3id!re:uen/" +earing loss, parti/ularl" 4+en +earing at 9(((6HA 4as 4orse t+an F(((6HA, +a5e an e;/ellent prognosis %((> o! patients in LairdGs series in %8F& re/o5ered /o3pletel" T+e 3a?orit" o! studies /on!ir3 t+e !indings t+at pro!ound +earing loss is a poor prognosti/ sign indi/ating 3ore se5ere in?ur"

onclusion
Sudden +earing loss is a 3edi/al /ondition 4+i/+ /an .e parti/ularl" de5astating to patients and !rustrating !or t+e otolar"ngologist to diagnose and treat Despite e;tensi5e in5estigation, onl" 3ini3al data +as .een generated in t+e past t+irt" "ears to i3pro5e our understanding o! t+e etiolog" and appropriate treat3ent o! t+is disease Most aut+orities agree t+at all patients s+ould undergo audio3etr", and i3aging 4it+ MRI !or t+ose patients 4it+ as"33etri/ +earing loss, +o4e5er t+e etiolog" !or t+e 3a?orit" o! patients 4ill go undiagnosed Treat3ent is 3ore /ontro5ersial Steroids +a5e .een s+o4n to signi!i/antl" i3pro5e +earing re/o5er" in patients 4it+ 3oderate to se5ere +earing loss and see3 to .e !a5ored !or t+e treat3ent o! autoi33une and idiopat+i/ !or3s o! SS)HL T+e re3ainder o! proposed treat3ents !or t+is disease are .ased, !or t+e 3ost part, on t+eor" and 4ill re:uire !urt+er in5estigation to /on!ir3 or dispro5e t+eir e!!i/a/"

&eferences
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sensorineural +earing loss Lar"ngos/ope %8F9M 89:==9D=== 1Ma"2 Pappas, Dennis G et al: Ultrastru/tural !indings in t+e /o/+lea o! aids /ases T+e #3eri/an $ournal o! Otolog" %889M %<:9<=D9=< Perl3an, H B and 7u3ura, R : E;peri3ental o.stru/tion o! t+e 5enousdrainage and arterial suppl" o! t+e inner ear #nn Otol R+inol Lar"ngol %8<EM ==:<&ED9= Sis3anis, # et$ al : Met+otre;ate t+erap" !o autoi33une +earing loss: # preli3inar" report Lar"ngos/ope %889M %(9:8&'D8&9 S/+e4eitAer, -anessa Ga"l and S+epard, )eil: Sudden +earing loss: #n un/o33on 3ani!estation o! 3ultiple s/lerosis Otolar"ngolog" Head and )e/6 Surger" %8F8M %((:&'ED&&' 1#pril2 S+i6o4itA, Mar6 $ : Sudden sensorineural +earing loss Medi/al Clini/s o! )ort+ #3eri/a %88%M E<:%'&8D%'<( 1)o5 2 S/+u6ne/+t, H , , Dono5an, E D : T+e pat+olog" o! idiopat+i/ sudden sensorineural +earing loss #r/+i5es o! Otolar"ngolog" %8F=M '9&:%D%< St Clair, E Cillia3 and M/Callu3, Re; M : CoganBs S"ndro3e Current Opinion in R+eu3atolog" %888M %%:9ED<' -asa3a, $ , Lint+i/u3, , H : Idiopat+i/ sudden sensorineural +earing loss: Te3poral .one +istopat+ologi/ stud" #nn Otol R+inol Lar"ngol '(((M %(8:<'ED<&' -eld3an, $an E , Hanada, Ta6e+iro, and Meeu4sen, ,rits: Diagnosti/ and t+erapeuti/ dile33as in rapidl" progressi5e sensorineural +earing loss and sudden dea!ness #/ta Otolar"ngol %88&M %%&:&(&D&(= -eltri, R C et$ al : T+e i3pli/ation o! 5iruses in idiopat+i/ sudden +earing loss: Pri3ar" in!e/tion or rea/ti5ation o! latent 5irusesN Otolar"ngolog" Head and )e/6 Surger" %8F%M F8:%&ED9% Cil6ins, S # $r , Matto;, D E , L"les, # : E5aluation o! a Js+otgunK gegi3en !or sudden +earing loss Otolar"ngolog" Head and )e/6 Surger" %8FEM 8E:9E9D9F( Cilson, Cillia3 R et$ al : T+e relations+ip o! idiopat+i/ sudden +earing loss to dia.etes 3ellitus Lar"ngos/ope %8F'M 8':%<<D%<8 1,e. 2 Cilson, C R et$ al : -iral and epide3iologi/ studies o! idiopat+i/ sudden +earing loss Otolar"ngolog" Head and )e/6 Surger" %8F&M 8%1=2:=<&DF Cilson, Cilli3a3 R , B"l, ,rederi/6 M , and Laird, )an: T+e e!!i/a/" o! steroids in t+e treat3ent o! idiopat+i/ sudden +earing loss #r/+i5es o! Otolar"ngolog" %8F(M %(=:EE'DEE= 1De/ 2