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Osseointegrated hearing device placement in congenital lamellar ichthyosis
Abby R Nolder1, Michael B Gluth
1Department of Otolaryngology-Head and Neck Surgery, University of Arkansas for Medical Sciences and Arkansas Children's Hospital, Little Rock, Arkansas 72205, USA.
Insights
Osseointegrated hearing devices offer a viable solution for children with congenital lamellar ichthyosis and conductive hearing loss. This case study shows successful device use with minimal skin reactions and improved hearing outcomes.
Area of Science:
- Otolaryngology
- Genetics
- Dermatology
Background:
- Congenital lamellar ichthyosis can cause conductive hearing loss due to external auditory canal stenosis.
- Osseointegrated hearing devices (OSSEO) are an option for hearing rehabilitation.
Observation:
- A 5-year-old female with congenital lamellar ichthyosis presented with bilateral external auditory canal stenosis and tympanic membrane blunting, leading to conductive hearing loss.
- Unilateral OSSEO placement was performed using a traditional skin flap technique.
Findings:
- Over 51 months, the patient experienced only minor skin reactions (Holgers Grade I) requiring minimal treatment.
- Aided speech reception threshold improved to 15 dB hearing level (HL) from 35 dB HL unaided.
- Continuous device usage was reported, with parental observation of improved school performance.
Implications:
- OSSEO placement appears to be a safe and effective option for hearing rehabilitation in children with congenital lamellar ichthyosis.
- This approach can overcome audiological challenges posed by skin-related comorbidities in this genetic condition.
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.
