Osseointegrated hearing device placement in congenital lamellar ichthyosis.

[lamellar ichthyosis]

To present a case of osseointegrated hearing device placement in a child with conductive hearing loss related to manifestations of congenital lamellar ichthyosis .A 5 -year -old female patient with congenital lamellar ichthyosis resulting in conductive hearing loss because of bilateral external auditory canal stenosis and tympanic membrane blunting .Unilateral osseointegrated hearing device placement using a traditional skin flap technique .Frequency and severity of adverse skin reactions , device usage , and audiometric testing .After 51 months of follow-up postoperatively , the patient has only required 2 treatments for minor skin reactions (Holgers Grade I ). Aided speech reception threshold was 15 dB hearing level (HL ) compared to 35 dB HL unaided . The subject has used the device continuously with parental report of improvement in school performance .Osseointegrated hearing device placement may be a viable option in patients with congenital lamellar ichthyosis despite the skin -related comorbidities known to be associated with this disease condition .