Laurel M. Fisher

Intratympanic steroid injection for treatment of idiopathic sudden hearing loss.

Objective. To conduct a clinical trial of intratympanic steroid injection for idiopathic sudden sensorineural hearing loss in subjects who failed oral steroid therapy. Study Design and Setting. Open-label methylprednisolone injection clinical trial in a tertiary neurotologic referral center. Twenty subjects (14 males; 6 females) received 4 injections within a 2-week period (4 days apart). Hearing, dizziness/p, and tinnitus were evaluated before and after treatment. Results. There were no serious unexpected adverse events and 2 types of expected adverse events (tympanic membrane perforation, nausea after injection). No increases in dizziness or tinnitus lasting longer than 24 hours were observed after injections. One of 20 (5%) improved to near-normal hearing. In addition, there was statistically significant improvement in 4-frequency pure-tone average and speech discrimination score at 1 month after treatment. Conclusion. Four intratympanic injections of methylprednisolone improved pure-tone average or speech discrimination scores for a subset of sudden hearing loss subjects that failed to benefit from oral steroids. Significance. A clinical trial of intratympanic injections for idiopathic sudden hearing loss was successfully completed and promising results were found.

Meniere's disease: prevalence of contralateral ear involvement.

Objective: Determine the prevalence and time interval for conversion from unilateral to bilateral involvement in Menieres disease and cochlear hydrops. Study Design and Setting: Retrospective chart review in a tertiary otologic referral center. Patients: 232 patients diagnosed with Menieres Disease (n = 186) or cochlear hydrops (n = 46) between 1959 and 2001, who visited the clinic over a five-year period between 1997-2001 and have at least 2 audiograms more than 12 months apart. Main Outcome Measures: Prevalence of cochlear hydrops relative to Menieres Disease, rate of progression from unilateral to bilateral involvement; interval between unilateral onset of symptoms and bilateral involvement; and rate of progression from cochlear hydrops to Menieres disease. Results: Initial diagnosis was Menieres disease in 71% and cochlear hydrops in 29% of all 950 hydropic patients presenting between 1997 and 2001. In the study sample, Menieres disease was bilateral at presentation in 11%; an additional 12% (14% of unilaterals) became bilateral during the follow-up period. At presentation, 6.5% of cochlear hydrops patients were bilateral, with another 26% becoming bilateral. Conversion from cochlear hydrops to Menieres disease occurred in 33% and some of these are included among the bilateral. The average time interval for conversion from unilateral to bilateral Menieres was 7.6 years (SD = 7.0 years). Conclusion: Most otologists are aware of the potential for contralateral ear involvement and conversion from cochlear hydrops to Menieres disease after diagnosis. These changes are significant, require long-term follow-up for detection, and may necessitate further treatment. Patients should be counseled regarding this potential when interventions are considered, especially with respect to ablative treatments.

Long-term hearing preservation after middle fossa removal of vestibular schwannoma.

OBJECTIVE: We sought to determine long-term hearing preservation in vestibular schwannoma patients after undergoing middle fossa resection. STUDY DESIGN, SETTING, AND OUTCOME MEASURES: We conducted a retrospective chart review of patients undergoing middle fossa resection from 1990 to 1995 at a tertiary care center. Pure-tone thresholds, before resection and at least 5 years after resection, and speech discrimination scores are reported. RESULTS: Seventy percent of patients with immediate postoperative hearing maintained serviceable hearing at more than 5 years after surgery. Pure-tone average in the operative ear changed at the same rate as hearing in the unoperated ear during this follow-up period. CONCLUSIONS: More than two thirds of patients who underwent middle fossa resection of a vestibular schwannoma with some hearing postoperatively maintain that hearing at greater than 5 years of follow-up. Surgery alone does not have a negative impact on long-term hearing preservation. (Otolaryngol Head Neck Surg 2003;129:660-5.)

Facial Nerve Function after Translabyrinthine Vestibular Schwannoma Surgery

OBJECTIVES: To evaluate the long-term facial function of patients after translabyrinthine vestibular schwannoma [VS] surgery and identify factors that influence these outcomes. STUDY DESIGN AND SETTING: A retrospective review was performed that included 580 consecutive patients who underwent translabyrinthine craniotomy for removal of VS at a tertiary referral neurotologic practice between February 2000 and July 2004. A total of 512 patients who underwent primary microsurgical treatment of sporadic unilateral VS met inclusion criteria. Patient and tumor characteristics as well as perioperative complications are described. Perioperative and long-term facial function were evaluated in 392 patients who had at least 1-year follow-up. RESULTS: Complication rates after translabyrinthine craniotomy for VS are low. Patients with smaller tumors have significantly better postoperative facial function than those with larger tumors. CONCLUSIONS: Excellent long-term facial function can be expected in the majority of patients who undergo microsurgical removal of VS via the translabyrinthine approach. Alternative treatment strategies may need to be developed for the treatment of VS > 3.5 cm in order to maximize postoperative facial function.

Vestibular schwannoma growth rates in neurofibromatosis type 2 natural history consortium subjects.

Objective: To determine the amount of growth in vestibular schwannomas in Neurofibromatosis type 2 (NF2) patients from diagnosis through short-term (up to 2 yr) and long-term (up to 4 yr) follow-up. Study Design: Retrospective magnetic resonance imaging (MRI) films were obtained on subjects enrolled in the NF2 Natural History study and examined for changes in vestibular schwannoma size over time. Setting: Data were collected from nine foreign and domestic NF2 centers, including hospital-based, academic, and tertiary care centers. Subjects: NF2 patients with MRI data and at least one follow-up examination within 9 months to 2 years of diagnosis were included; n = 56 patients with 84 lesions for evaluation of growth. Intervention: Routine, clinically obtained, magnetic resonance images were digitized and measured using image management software. Short-term follow-up was defined as up to 2 years (n = 84 lesions), and long-term follow-up was defined as 3 to 4 years (n = 29 lesions). Outcome Measures: Vestibular schwannoma size was assessed using anterior–posterior, medial–lateral, and greatest diameter linear measurements. Results: Vestibular schwannomas increased in size (at least 5 mm) in 8% of the vestibular schwannomas across short-term follow-up. At long-term follow-up, 13% of the tumors had increased in size. On average, schwannomas increased in greatest diameter 1.3 mm per year across short-term follow-up. Conclusion: Slightly greater than 1 in 10 diagnosed NF2-related vestibular schwannomas increased in size by at least 5 mm by 4 years of follow-up, if still untreated at that time.

Oral steroid regimens for idiopathic sudden sensorineural hearing loss.

OBJECTIVE To determine hearing recovery in patients with idiopathic sudden hearing loss treated with varying amounts of oral steroids. STUDY DESIGN AND SETTING A retrospective chart review (n = 75) in a tertiary care clinic examined sudden hearing loss patients treated with 1 60-mg prednisone taper, 1 course of steroid less than a 60-mg taper, or any 2 courses of oral steroid. RESULTS: Overall, 35% of the patients recovered a clinically significant amount of hearing. Recovery was associated with immediate treatment (within 2 weeks from onset), better hearing at the onset of treatment, and treatment with the higher dose of prednisone in patients with just 1 additional symptom (dizziness or tinnitus). Patients tended to continue to experience some recovery in hearing up to 4 months after treatment. CONCLUSION: Immediate treatment of patients with unilateral idiopathic sudden hearing loss and additional symptoms (dizziness or tinnitus) with a 14-day course of 60 mg prednisone (with taper) is recommended. EBM rating: B-3.

Immunohistochemistry study of human vestibular nerve schwannoma differentiation

Differentiation of primary human vestibular nerve schwannomas (VS) caused by mutations of the NF2 gene was evaluated by examining the expression patterns of genes that are specifically expressed in different stages of Schwann cell lineage. In schwannoma cells that are not in contact with an axon, the expression levels of the major myelin sheath proteins, such as protein zero glycoprotein (P0), myelin basic protein (MBP), and peripheral myelin protein 22 (PMP22), were greatly reduced. However, high expression levels of nerve growth factor receptor (NGFR), neural cell adhesion molecule (N‐CAM), and cell adhesion molecule L1 (L1) were observed. In addition, expression of transcription factors Krox20, Krox24, and SCIP/Oct6 was also detected in the tumor cells. These results suggest that loss of the NF2 gene was responsible for the transformation of the Schwann cells into a neoplastic stage that has a similar genetic profile to the pro‐myelinating stage. Finally, the primary human vestibular schwannoma cells failed to be regulated and redifferentiated by a regenerating axon, when the human tumors were transplanted into sciatic nerve of nude rat. These results suggest that the NF2 gene might be involved in the differentiation of Schwann cells. GLIA 38:363–370, 2002.

SAS-CIS preference study in postlingually deafened adults implanted with the CLARION cochlear implant.

This study examined the speech perception performance of 71 postlingually deafened adults implanted with the CLARION® Multi-Strategy™ Cochlear Implant. After 3 months of implant use, one third of the patients (n = 23) preferred to use the Simultaneous Analog Stimulation (SAS) strategy, and two thirds of them (n = 48) chose to use the Continuous Interleaved Sampler (CIS) strategy. The mean CID sentence scores were similar for the SAS and CIS user groups at the 3-month postoperative interval. In contrast, the mean CNC word score of the SAS users was significantly higher than the mean score of the CIS users after 3 months of implant experience. Patients who preferred to use SAS demonstrated significantly shorter duration of deafness at time of implantation than did the CIS users, whereas the CIS users demonstrated significantly higher preoperative sentence recognition scores than the SAS users. The results suggested that access to a preferred processing strategy improved the overall group performance of postlingually deafened adults in the present study compared to the results of previous studies in which only the CIS strategy was available to most patients with the Clarion.

The effect of long-term deafness on speech recognition in postlingually deafened adult CLARION cochlear implant users.

Research suggests that duration of deafness prior to cochlear implantation affects postoperative speech recognition in adults. Duration of deafness usually is defined as the number of years between the onset of profound deafness and implantation. The purpose of this study was to examine the effects of duration of deafness — expressed as percentage of life with deafness — independent of age at implantation on postoperative speech recognition in adult postlingually deafened CLARION® Multi-Strategy™ Cochlear Implant users. Speech recognition tests (CID sentences and NU6 words) were administered to 202 consecutively implanted adults preoperatively and at 3 and 6 months after initial device fitting. Patients implanted at a younger age and those with smaller percentages of their lives with deafness achieved the highest levels of short-term postoperative speech recognition. Patients who had been deaf for ≥60% of their lives demonstrated a slower rate of speech recognition improvement than those with shorter durations of deafness, but still continued to improve with increased implant experience.

The relationship between high-frequency pure-tone hearing loss, hearing in noise test (HINT) thresholds, and the articulation index.

BACKGROUNDnSpeech recognition in noise testing has been conducted at least since the 1940s (Dickson et al, 1946). The ability to recognize speech in noise is a distinct function of the auditory system (Plomp, 1978). According to Kochkin (2002), difficulty recognizing speech in noise is the primary complaint of hearing aid users. However, speech recognition in noise testing has not found widespread use in the field of audiology (Mueller, 2003; Strom, 2003; Tannenbaum and Rosenfeld, 1996). The audiogram has been used as the gold standard for hearing ability. However, the audiogram is a poor indicator of speech recognition in noise ability.nnnPURPOSEnThis study investigates the relationship between pure-tone thresholds, the articulation index, and the ability to recognize speech in quiet and in noise.nnnRESEARCH DESIGNnPure-tone thresholds were measured for audiometric frequencies 250-6000 Hz. Pure-tone threshold groups were created. These included a normal threshold group and slight, mild, severe, and profound high-frequency pure-tone threshold groups. Speech recognition thresholds in quiet and in noise were obtained using the Hearing in Noise Test (HINT) (Nilsson et al, 1994; Vermiglio, 2008). The articulation index was determined by using Pavlovics method with pure-tone thresholds (Pavlovic, 1989, 1991).nnnSTUDY SAMPLEnTwo hundred seventy-eight participants were tested. All participants were native speakers of American English. Sixty-three of the original participants were removed in order to create groups of participants with normal low-frequency pure-tone thresholds and relatively symmetrical high-frequency pure-tone threshold groups. The final set of 215 participants had a mean age of 33 yr with a range of 17-59 yr.nnnDATA COLLECTION AND ANALYSISnPure-tone threshold data were collected using the Hughson-Weslake procedure. Speech recognition data were collected using a Windows-based HINT software system. Statistical analyses were conducted using descriptive, correlational, and multivariate analysis of covariance (MANCOVA) statistics.nnnRESULTSnThe MANCOVA analysis (where the effect of age was statistically removed) indicated that there were no significant differences in HINT performances between groups of participants with normal audiograms and those groups with slight, mild, moderate, or severe high-frequency hearing losses. With all of the data combined across groups, correlational analyses revealed significant correlations between pure-tone averages and speech recognition in quiet performance. Nonsignificant or significant but weak correlations were found between pure-tone averages and HINT thresholds.nnnCONCLUSIONSnThe ability to recognize speech in steady-state noise cannot be predicted from the audiogram. A new classification scheme of hearing impairment based on the audiogram and the speech reception in noise thresholds, as measured with the HINT, may be useful for the characterization of the hearing ability in the global sense. This classification scheme is consistent with Plomps two aspects of hearing ability (Plomp, 1978).