Investigation of Endolymphatic Hydrops by Electrocochleography in Patients with Cogan's Syndrome

Abstract

We compared auditory and vestibular function between a patient with typical Cogan's syndrome and a patient with atypical Cogan's syndrome. Repeat audiograms demonstrated fluctuating sensorineural hearing loss in the affected ear. Brainstem auditory evoked response testing revealed no abnormalities. Hallpike caloric testing showed a decreased response in the affected ear in the typical case and responses within normal limits in the atypical case. Sinusoidal harmonic acceleration was normal in the typical case, and there was an abnormal phase at 0.01 Hz in the atypical case.

Endolymphatic hydrops was investigated by electrocochleography. In both patients, there was an enhancement of the summating potential, with an increased ratio of summating potential to action potential amplitude. This finding is consistent with endolymphatic hydrops.

References

Cogan

D.G.

Syndrome of nonsyphilitic Keratitis and Vestibulo-auditory symptoms. Arch Ophthalmol 1945; 33: 144–9.

Cody

D.T.

, Williams

H.L.

Cogan's syndrome. Laryngoscope 1960; 70: 447–78.

Haynes

B.F.

, Kaiser-Kupfer

M.I.

, Mason

, Fauci

A.S.

Cogan syndrome: Studies in thirteen patients, long-term follow-up, and a review of the literature. Medicine (Baltimore) 1980; 59: 426–41.

Vollertsen

R.S.

, McDonald

T.J.

, Younge

B.R.

Cogan's syndrome: 18 cases and a review of the literature. Mayo Clin Proc 1986; 61: 344–61.

Benitez

J.T.

, Arsenault

M.D.

, Licht

J.M.

Evidence of central vestibulo-auditory dysfunction in atypical Cogan's syndrome: A case report. Am J Otol 1990; 11: 131–4.

Arsenault

M.D.

, Benitez

J.T.

Electrocochleography: A method for making the Stypulkowski-Staller electrode and testing technique. Ear Hear 1991; 12: 358–60.

Eggermont

J.J.

Summating potentials in electrocochleography: Relation to hearing disorders. In: Ruben

R.J.

, Elberling

, Salomon

, eds. Electrocochleography. Baltimore: University Park Press 1976: 67–87.

Coats

A.C.

The summating potential and Ménière's disease. I. Summating potential amplitude in Ménière and non-Ménière ears. Arch Otolaryngol 1981; 107: 199–208.

Gibson

W.P.

, Prasher

D.K.

, Kilkenny

G.P.

Diagnostic significance of transtympanic electrocochleography in Ménière's disease. Ann Otol Rhinol Laryngol 1983; 92: 155–9.

10.

Atkinson

Ménière's original papers. Acta Otolaryngol Suppl (Stockh) 1961; 162: 1–78.

11.

McCabe

B.F.

Autoimmune sensorineural hearing loss. Ann Otol Rhinol Laryngol 1979; 88: 585–9.

12.

McCabe

B.F.

Autoimmune inner ear disease. Presented at the University of Minnesota's Third International Symposium on Clinical Otology, 1981, Minneapolis.

13.

Hughes

G.B.

, Kinney

S.E.

, Barna

B.P.

Autoimmune reactivity in Cogan's syndrome: A preliminary report. Otolaryngol Head Neck Surg 1983; 91: 24–32.

14.

Aran

J.M.

, Rarey

K.E.

, Hawkins

J.E.

Jr. Functional and morphological changes in experimental endolymphatic hydrops. Acta Otolaryngol (Stockh) 1984; 97: 547–57.

15.

Gibson

W.P.

The use of electrocochleography in the diagnosis of Ménière's disease. Acta Otolaryngol Suppl (Stockh) 1991; 485: 46–52.

16.

Arenberg

I.K.

, Obert

A.D.

, Gibson

W.P.

Intraoperative electrocochleographic monitoring of inner ear surgery for endolymphatic hydrops: A review of cases. Acta Otolaryngol Suppl (Stockh) 1991; 485: 53–64.

17.

Fisher

E.R.

, Hellstrom

H.R.

Cogan's syndrome and systemic vascular disease: Analysis of pathologic features with reference to its relationship to thromboangiitis obliterans (Buerger). Arch Pathol 1961; 72: 572–92.

18.

Wolff

, Bernhard

W.G.

, Tsutsumi

The pathology of Cogan's syndrome causing profound deafness. Trans Am Otol Soc 1965; 53: 94–109.

19.

Rarey

K.E.

, Bicknell

J.M.

, Davis

L.E.

Intralabyrinthine osteogenesis in Cogan's syndrome. Am J Otolaryngol 1986; 7: 387–90.