Bilateral cochlear implantation in a patient with osteopathia striata voorhoeve and cranial sclerosis

Objective: Osteopathia Striata with Cranial Sclerosis (OS-CS) is a rare form of X-linked dominant sclerosing bone dysplasia. The estimated prevalence of OS-CS is less than 1 in 1,000,000 in the general population with a little over 100 reported cases so far. Up to half of the patients suffer from hearing loss. Hearing loss is usually conductive rather than mixed or sensorineural in patients with OS-CS sclerosis. Herein, we report our experience with a patient who although initially had a conductive hearing loss, later developed sensorineural hearing loss on both sides and was treated with bilateral cochlear implantation. Patient: Thirteen-year-old female patient suffering from Osteopathia striata with cranial sclerosis and functional deafness in the left ear and a high-grade sensorineural hearing loss in the right ear. Intervention: Bilateral cochlear implantation Main outcome measures: Postoperative hearing gain, clinical outcome, audiological examination. Results: The patient showed good hearing gain of 30 dB on the left side and between 30 dB and 40 dB on the right side. Conclusions: Despite the cranial sclerosis, cochlear implantation is a viable option in improving life quality of patients with Osteopathia striata. Nevertheless, it should be noted that in such cases, there is a possibility of progressive sclerosis leading to further narrowing of the internal auditory canal or even complete closure thereby damaging the auditory nerve irreversibly.