Osseointegrated hearing device placement in congenital lamellar ichthyosis.

OBJECTIVE

To present a case of osseointegrated hearing device placement in a child with conductive hearing loss related to manifestations of congenital lamellar ichthyosis.

PATIENT: 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.

INTERVENTION: Unilateral osseointegrated hearing device placement using a traditional skin flap technique.

MAIN OUTCOME MEASURES: Frequency and severity of adverse skin reactions, device usage, and audiometric testing.

RESULTS

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.

CONCLUSION

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.