XLIV The Pathology of Cogan's Syndrome Causing Profound Deafness

Abstract

Exactly twenty years ago, David Cogan of Harvard University, Department of Ophthalmology, reported four cases of "Nonsyphilitic Interstitial Keratitis Associated with Vestibulo-auditory Symptoms." All four cases came to him within a year, all from the environs of Boston. In his cases the corneal changes progressed rela tively little but the vestibular condition incapacitated the patients for several weeks or months and the auditory state led to profound deafness.Cogan discussed the differences between this disease and syphilitic keratitis, noting that in the latter condition, only 4% of the cases developed deafness and the deafness did not occur until months or years after the keratitis. Dr. Cogan and his associates have continued to report on these cases of non-syphilitic keratitis with vestibular and cochlear symptoms, as in 1949 when the case load had risen to 13, 1959 when two deaths but no autopsies were reported and 1963 when a limited autopsy on a 14 year-old boy was obtained.Reports of approximately 36 of these cases have come to our attention. They mostly concern relatively young people, although the age range is now ΐΥζ years to 60. Only one Negro and one Cuban are in the list. Reports have been in the Italian and Swiss literature. The disease was not listed on the program of a recent symposium in Philadelphia on vestibular diseases, October, 1964.

Cogan's Syndrome Treated With Oral Glycerine: Report of a Case

“…In addition, they listed other signs of generalized disease in these patients as follows: diarrhea, 3; leukocyto¬ sis, 18; respiratory tract infection, 15; ner¬ vous system symptoms, 12; musculoskeletal, 11; gastrointestinal tract symptoms, 10; fe¬ ver, 9; cardiovascular symptoms, 8; geni¬ tourinary infection, 7; and lymphadenopathy, 6. Only five of the 27 had no other symptoms other than those referrable to the eye or ear.…”

“…In the following case, the patient obtained a dramatic gain in hearing on two occasions following oral administration of glycerin, 1.5 gm/kg. The rationale for this therapy was based on a description of the pathology of Cogan's syndrome by Wolff et al 6 In an autopsy specimen, Wolff and associates described the pathology of Co¬ gan's syndrome as that of fibrous tissue and new bone growth in the perilymphatic spaces, and hydrops involving the saccule and cochlear duct. It must be noted, how¬ ever, that the case reported had a clinical picture of hearing loss, pigment changes in the lashes, exudative retinal detachment, and loss of hair.…”

Histopathology of the Human Inner Ear in the Cogan Syndrome with Cochlear Implantation

“…In the other case, an abrupt deterioration of loudness perception required an increase in electrical stimulation. Generally, new bone formation is seen in the cochlea after cochlear implantation for deafness of various etiologies [Li et al, 2007; Somdas et al, 2007; Seyyedi and Nadol, 2014; Kamakura and Nadol, 2016], as well as in the cochleae deafened by Cogan’s syndrome without cochlear implantation [Fisher and Hellstrom, 1961; Wolff et al, 1965; Zechner, 1980; Rarey et al, 1986; Schuknecht and Nadol, 1994]. Postoperative word recognition scores have been reported to be negatively correlated with the % volume of new bone formation within the cochlea after implantation [Kamakura and Nadol, 2016].…”

“…Although there have been several reports of the histopathology of the inner ear in Cogan’s syndrome [Fisher and Hellstrom, 1961; Wolff et al, 1965; Zechner, 1980; Rarey et al, 1986; Schuknecht and Nadol, 1994; Ishii et al, 1995; Jung et al, 2016] to date there has been no description of the histopathology of the inner ear following cochlear implantation in Cogan’s syndrome. The temporal bones of a 64-year old patient who suffered from a bilateral onset of rapidly progressive and profound sensorineural hearing loss due to Cogan’s syndrome and who underwent bilateral cochlear implantation 19 months prior to death have been obtained by the Otopathology Laboratory of the Massachusetts Eye and Ear Infirmary.…”

Cogan Syndrome: The Audiological Picture

“…13 The stapes and the vestibulostapedial joint were found to be nor¬ mal. However, some doubt exists as to the diagnosis of this case, whether it was a Cogan syndrome or a VogtKoyanagi-Harada syndrome.14 There¬ fore, in typical cases of Cogan syn¬ drome, histological examination of middle ear structures, especially the stapes and the vestibulostapedial joint, has to be carried out in order to unveil the mechanism underlying the middle ear dysfunction.…”