Spectrum of Third Window Abnormalities: Semicircular Canal Dehiscence and Beyond.

Third window abnormalities are defects in the integrity of the bony structure of the inner ear, classically producing sound-/pressure-induced vertigo (Tullio and Hennebert signs) and/or a low-frequency air-bone gap by audiometry. Specific anatomic defects include semicircular canal dehiscence, perilabyrinthine fistula, enlarged vestibular aqueduct, dehiscence of the scala vestibuli side of the cochlea, X-linked stapes gusher, and bone dyscrasias. We discuss these various entities and provide key examples from our institutional teaching file with a discussion of symptomatology, temporal bone CT, audiometry, and vestibular-evoked myogenic potentials.

Evaluation of a behavioral audiometry simulator for teaching visual reinforcement audiometry.

Eight audiology students with little or no visual reinforcement audiometry (VRA) experience each performed a VRA test on an infant. Four of the students received approximately 5 hours of behavioral audiometry simulator (BAS) training, and 1 week later, after the first VRA test, all eight students tested a second infant. Student performance was rated by three audiologists who were experienced in performing VRA with infants. The performance of the group that received BAS training improved significantly while the performance of the control group did not. Among the students who received BAS training, those who improved the most during stimulated testing also showed the greatest improvement in VRA with real infants.

Infant auditory temporal acuity: gap detection.

The development of auditory temporal acuity during infancy was examined in 3-, 6-, and 12-month-old infants and in adults using the gap detection paradigm. Listeners detected a series of gaps, or silent intervals, or variable duration in a broadband noise. In order to vary the acoustic frequencies available to the listener, a high-pass noise was used to mask frequencies above specified cutoffs. High-pass maskers with cutoffs of 500, 2,000, and 8,000 Hz were used. The minimum detectable gap was determined using the Observer-based Psychoacoustic Procedure. The thresholds of 3- and 6-month-olds were considerably poorer than those of the adults, although the effect of masker condition was about the same for these 3 groups. The thresholds of 12-month-olds were significantly worse than the adults when the stimulus was unmasked or when the masker cutoff frequency was 2,000 or 8,000 Hz. When the masker cutoff frequency was 500 Hz, 12-month-olds fell into 2 groups: some had gap thresholds that were about the same as 3- and 6-month-olds, while some had gap thresholds that approached those of adults. In a second experiment, a larger group of 12-month-olds were tested with a 500-Hz masker cutoff. Average performance of 12-month-olds was about the same as that of 3- and 6-month-olds in Experiment 1. Some infants attained thresholds close to those of adults. Thus, gap detection thresholds are quite poor in infants, although the similarity of the effect of frequency on performance in infants and adults suggests that the mechanisms governing temporal resolution in infants operate qualitatively like those in adults.

Promontory electrical stimulation in labyrinthectomized ears.

Bilateral deafness can occur in patients with Menière's disease who have undergone a labyrinthectomy in one ear. To investigate the feasibility of a cochlear implant in the labyrinthectomized ear, promontory electrical testing by transtympanic needle was performed in six patients who had undergone a unilateral transmastoid labyrinthectomy 6 weeks to 5 years previously. All patients had a behavioral response to the stimulus, and each described a different pitch percept with the four frequencies used. Five of the patients demonstrated an electrically evoked middle latency response. These data are comparable with behavioral and electrophysiologic responses from ears deafened by other causes and now successfully implanted. The results suggest that peripheral neural elements and central auditory pathways remain at least partially functional many years after a labyrinthectomy. Thus, a labyrinthectomy should not be withheld as a surgical option if otherwise indicated.

Pure-tone sensitivity of human infants.

Pure-tone thresholds at frequencies ranging from 250 to 8000 Hz were estimated for 3-, 6-, and 12-month-old infants and for adults, using the Observer-based Psychoacoustic Procedure (OPP). Sounds were presented monaurally using an earphone. Psychometric functions of infants were similar to those of adults, although 3-month-olds had shallower functions at higher frequencies. The thresholds of 6- and 12-month-old infants were 10-15 dB higher than those of the adults, with the difference being greater at lower frequencies. This result is in general agreement with results from other laboratories. The thresholds of 3-month-olds were 15-30 dB higher than those of adults. The greatest difference between 3-month-olds and adults was at 8000 Hz. This threshold difference is smaller than that reported in earlier behavioral studies; higher thresholds at high frequencies have been previously reported for newborn and 3-month-old infants. The relative contributions of sensory and nonsensory variables to these age differences are discussed.

Level and age effects in infant frequency discrimination.

Frequency difference limens (FDLs) were estimated for 3-, 6-, and 12-month-old infants and for adults using pure tones at 500, 1000, and 4000 Hz. Each listener provided an FDL at 40 dB and at a higher (80 dB, in most cases) sensation level (SL). An observer-based behavioral testing technique was used. The FDLs of 3-month-olds were worse than those of adults at all three frequencies, and increased with increasing frequency. The FDLs of 6- and 12-month-olds were worse than those of adults at 500 and 1000 Hz, but not at 4000 Hz. Decreasing the SL led to an increase in the FDL of about the same magnitude at all ages, and the same age differences were found at both SLs. Thus infant-adult differences in FDL are not a simple consequence of differences in absolute sensitivity. Infant FDLs at one SL were also found to be significantly correlated with the FDL at the other SL. The FDLs at one age were, in general, predictive of the FDL at a later age in a longitudinal sample of infants. Models that might account for these age-related differences are discussed.