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Auditory Manifestations of Keratitis‐Ichthyosis‐Deafness (KID) Syndrome
Author(s) -
SzymkoBennett Yvonne M.,
Russell Laura J.,
Bale Sherri J.,
Griffith Andrew J.
Publication year - 2002
Publication title -
the laryngoscope
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.181
H-Index - 148
eISSN - 1531-4995
pISSN - 0023-852X
DOI - 10.1097/00005537-200202000-00014
Subject(s) - medicine , audiology , audiogram , hearing loss , sensorineural hearing loss , audiometry , auditory neuropathy , dermatology
Objective Evaluation of the auditory manifestations of Keratitis‐Ichthyosis‐Deafness (KID) syndrome, a rare genodermatosis characterized by follicular hyperkeratosis, vascularizing keratitis, and congenital hearing loss. Study Design Five individuals with sporadic KID syndrome were evaluated in the outpatient audiology clinic at the Warren Grant Magnuson Clinical Center of the National Institutes of Health. Methods Audiologic examinations included pure‐tone audiometry, speech audiometry, and middle ear immittance testing. Auditory brainstem responses and otoacoustic emissions were analyzed in 2 subjects. Results Four subjects had prelingual, bilateral, profound sensorineural hearing loss, whereas the fifth subject had significant residual hearing that exhibited no progression on serial audiograms. All 5 subjects had a history of non‐erosive keratosis obturans and cutaneous cysts in the external ear canals that prevented continuous use of ear molds. Conclusions The sensorineural hearing loss in KID syndrome is generally prelingual and profound. This combination of auditory and cutaneous phenotypes is similar to those previously reported for KID syndrome. KID syndrome presents a difficult challenge for communication rehabilitation because keratitis may impair the perception of sign and spoken language, and the cutaneous manifestations routinely curtail use of external amplification devices.