Case report of auditory canal obstruction in congenital infiltrating lipomatosis of the face

Background  Congenital infiltrating lipomatosis of the face (CILG) is a rare non-hereditary disease associated with soft-tissue, skeletal, and dental anomalies. Patients often suffer from hypertrophy of the soft tissues of the face, malfunctions in the area of ​​the temporomandibular joint, mucosal neuromas and visual problems. Surgerys are often associated with a high risk of recurrence. This case report describes a patient who developed symptomatic ear canal stenosis during CILG. Until now such a manifestation has not been described in the literature.

Case Report  A 22-year-old patient presented with a history of progressive otalgia and hearing loss on the right side for about two weeks. Since birth, she has suffered from facial dysmorphia with suspected CILG. Alio loco she had already undergone various liposuction and dental operations since childhood. In the ENT medical examination, a noticeable stenosis of the auditory canal appeared on the right side. The eardrum was not visible. Pure tone audiometry revealed conductive hearing loss on the right side with normal acusis on the left. We therefore performed an enlargement of the external auditory canal. The patient did not want any further interventions, particularly those of cosmetic nature. Postoperatively, there was a significant improvement in the otalgia and an improvement in hearing.

Summary We report on a CILG-patient who, in addition to the typical facial asymmetry, developed an obstruction of the ear canal with otalgia and hearing loss. We therefore performed an ear canal enlargement plastic surgery, which resulted in an improvement in the otalgia and the hearing loss. If a symptomatic ear canal stenosis occurs, an enlargement of the ear canal should be offered or performed despite the high risk of recurrence.

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Artikel online veröffentlicht:
12. Mai 2023