Temporal Bone Disorders in Children

Hearing Loss in Malignant Infantile Osteopetrosis: A Case-Based Review

“…It is usually located below the floor of hypotympanum and separated from the middle ear by a layer of bone. [21][22][23][24][25][26][27] In 0.5%-1.7%, this bony septum is absent which is defined as a dehiscent jugular bulb. 23,24 Consequently, the jugular bulb can expand and protrude into the middle ear, which can be noticed during otoscopy as a red-purplish mass in the posterior-inferior quadrant of the tympanic membrane.…”

“…Both conductive hearing loss and pulsatile tinnitus are associated with a dehiscent jugular bulb. [22][23][24][25][27][28][29] Impinging of the dehiscent jugular bulb on the tympanic membrane, middle ear ossicles, or round window can cause conductive hearing loss. 24,26,27 Furthermore, this important anatomic variation can entail significant morbidity and even mortality during middle ear surgery.…”

“…Several cases of jugular injury and massive bleeding, in particular during myringotomy and tympanomeatal flap elevation, have been reported in the past. 25,27,28 In current literature, the etiology of jugular bulb anomalies is poorly understood. Moreover, there is no evidence of a dehiscent jugular bulb associated with osteopetrosis.…”

“…Several cases of jugular injury and massive bleeding, in particular during myringotomy and tympanomeatal flap elevation, have been reported in the past. 25 , 27 , 28 …”