Pediatric fluctuating sensorineural hearing loss: problems in medical management.

OBJECTIVES: To discuss the diagnosis and management of children with fluctuating sensorineural hearing loss, especially focusing on those problems dealing with autoimmune inner ear disease. STUDY DESIGN: A retrospective chart review of a large pediatric otolaryngology practice. A series of 40 children with progressive hearing losses was identified. Of that group, 22 children, aged 1.5 to 12.2 years at first audiogram, were considered to have fluctuating sensorineural hearing loss (FSNHL). Criteria for inclusion in the FSNHL group were threshold variations of 15 dB or more in at least one ear at two or more of the standard audiometric frequencies on at least 2 testing days. METHODS: Charts were reviewed for age, sex, otologic history, laboratory evaluations, medical or surgical treatments, significant medical history, and family medical history. RESULTS: Twenty-two children met the criteria for fluctuating sensorineural hearing loss. Of those with fluctuating hearing loss, 15 were idiopathic, 3 had positive lymphocyte transformation tests (LTT) suggestive of autoimmune inner ear disease (AIED), and 4 had fistula on middle ear exploration. Average fluctuation for all groups was 29.1 dB. Average duration of fluctuations was 4.95 years. CONCLUSIONS: The majority of pediatric FSNHL cases (15 of 22) were idiopathic in nature. Of those FSNHL children with positive LTTs, only one was treated with steroid therapy. In the other patients with positive LTTs, parents or other physicians were often reluctant to treat, or the patient was lost to follow-up. Mean fluctuations varied substantially across all standard audiometric frequencies for all groups.

Normal audiologic presentations in patients with acoustic neuroma: An evaluation using strict audiologic parameters.

Although several studies have previously reported on patients presenting with "normal" audiologic parameters in acoustic neuroma, the present study is, to our knowledge, the first to exclusively examine in detail cases involving exceptionally stringent objective audiometric features. Of 369 patients with acoustic neuroma who were operated on between April 1980 and April 1997 by our group, 10 had strictly normal hearing, defined as follows: (1) pure-tone average < 20 dB; (2) speech discrimination score > 90%; and (3) interaural differences

Complications of gold weight eyelid implants for treatment of fifth and seventh nerve paralysis.

Complications occurred in six patients after gold weights were implanted into the upper eyelid tissues for fifth and seventh nerve palsies. These complications included implant infection without extrusion (in one patient); entropion with trichiasis and presumed inflammatory reaction to the gold weight material (in one patient); upper eyelid distortion and poor eyelid contour with corneal ulceration and scarring (in one patient); significant residual lagophthalmos with exposure keratitis (in one patient); and blepharoptosis obscuring the pupillary access (in two patients). Resolution of the complications required 1. implant removal in four of six patients without reinsertion of a second weight, 2. recession of the retractors of the upper eyelids with medial and lateral canthoplasty (in four patients), and 3. permanent tarsorrhaphy (in one patient). The authors conclude that complications may be minimized by careful preoperative determination of the optimum implant size, weight, and placement within the eyelid as well as meticulous attention to the surgical technique of implantation. The use of other eyelid protective procedures is often necessary to augment corneal protection especially in patients with combined fifth and seventh cranial nerve palsies. Endogenous implant infection without extrusion of the gold weight may be distinguished from presumed inflammation due to gold allergy by clinical response to antibiotics in the former and requirements of steroids or removal of the implant in the latter.

Rethinking the use of auditory brainstem response in acoustic neuroma screening.

The ability of magnetic resonance imaging (MRI) to detect very small acoustic tumors has triggered many to rethink the use of auditory brainstem response (ABR) in the screening of acoustic tumors. To assess ABR accuracy, we conducted a retrospective study of 388 surgically treated patients. Of these patients, 111 had complete databases including both preoperative MRIs and ABRs. The ABR was abnormal by wave V interaural latency difference in 106 (95%) of the cases. Although our overall sensitivity was 95%, sensitivity varied according to tumor size. ABR was abnormal or absent for all tumors (100%) larger than 2 cm in diameter, for 98% of tumors 1.1 to 2 cm in diameter, and for only 89% of tumors less than or equal to 1 cm in diameter. Ramifications of this in the decision-making process are presented. Criteria for cut-off values are also discussed.