Characteristic findings in the human fetus vestibule: A human temporal bone study.

OBJECTIVE: The "collapse," a highly flexed, dented, or caved membrane between the endo- and peri-lymph of the saccule and utricle in adults, is considered as a morphological aspect of Ménière's syndrome. Likewise, when mesh-like tissues in the perilymphatic space are damaged or lost, the endothelium loses mechanical support and causes nerve irritation. However, these morphologies were not examined in fetuses. METHODS: By using histological sections from 25 human fetuses (crown-rump length[CRL] 82-372 mm; approximately 12-40 weeks), morphologies of the perilymphatic-endolymphatic border membrane and the mesh-like tissue around the endothelium were examined. RESULTS: The highly flexed or caved membrane between the endo- and peri-lymphatic spaces was usually seen in the growing saccule and utricle of fetuses, especially at junctions between the utricle and ampulla at midterm. Likewise, the perilymphatic space around the saccule, utricle and semicircular ducts often lost the mesh-like tissues. The residual mesh-like tissue supported the veins, especially in the semicircular canal. CONCLUSION: Within a cartilaginous or bony room showing a limited growth in size but containing increased perilymph, the growing endothelium appeared to become wavy. Owing to a difference in growth rates between the utricle and semicircular duct, the dentation tended to be more frequently seen at junctions than at free margins of the utricle. The difference in site and gestational age suggested that the deformity was not "pathological" but occurred due to unbalanced growth of the border membrane. Nevertheless, the possibility that the deformed membrane in fetuses was an artifact caused by delayed fixation is not deniable.

Fatty acid binding protein type 7 deficiency preserves auditory function in noise-exposed mice.

Fatty acid-binding protein 7 (FABP7) is vital for uptake and trafficking of fatty acids in the nervous system. To investigate the involvement of FABP7 in noise-induced hearing loss (NIHL) pathogenesis, we used Fabp7 knockout (KO) mice generated via CRISPR/Cas9 in the C57BL/6 background. Initial auditory brainstem response (ABR) measurements were conducted at 9 weeks, followed by noise exposure at 10 weeks. Subsequent ABRs were performed 24 h later, with final measurements at 12 weeks. Inner ears were harvested 24 h after noise exposure for RNA sequencing and metabolic analyses. We found no significant differences in initial ABR measurements, but Fabp7 KO mice showed significantly lower thresholds in the final ABR measurements. Hair cell survival was also enhanced in Fabp7 KO mice. RNA sequencing revealed that genes associated with the electron transport chain were upregulated or less impaired in Fabp7 KO mice. Metabolomic analysis revealed various alterations, including decreased glutamate and aspartate in Fabp7 KO mice. In conclusion, FABP7 deficiency mitigates cochlear damage following noise exposure. This protective effect was supported by the changes in gene expression of the electron transport chain, and in several metabolites, including excitotoxic neurotransmitters. Our study highlights the potential therapeutic significance of targeting FABP7 in NIHL.

Functional hearing loss and developmental imbalances.

OBJECTIVE: Functional hearing loss (FHL) is a disorder in which there are abnormal values on a hearing test, despite the absence of organic abnormalities in the peripheral and central auditory pathways. Here, we examined the developmental characteristics of FHL and the importance of intervention by analyzing the clinical characteristics of children with this disorder. METHODS: We retrospectively examined 16 patients assessed under a diagnosis of FHL. After interventions such as psychological counseling by our pediatrics and psychiatry departments, we compared the clinical profiles of patients in which hearing was "improved/normalized" and "unimproved". RESULTS: Fourteen patients visited a pediatrician and two chose not to do so. A discrepancy between the maximum and minimum values of the four index scores was observed in all patients in which WISC-IV (the fourth version of the Wechsler Intelligence Scale for Children) was performed (n = 12). The discrepancy between the verbal comprehension index (VCI) and perceptual reasoning index (PRI) was significantly greater in "unimproved" patients than in "improved/normalized" patients. Hearing improved, or was normalized, after intervention in six of 16 patients. CONCLUSIONS: Developmental imbalances were suspected in all 12 children who visited a pediatrician and completed the WISC-IV. Cooperation with pediatricians, psychiatrists, and other health professionals is desirable in supporting patients diagnosed with FHL.

Transient connection between the vestibular aqueduct and utricle: A study using sagittal sections of human embryonic heads.

BACKGROUND: The aqeductus vestibuli (aqueduct) is believed to connect to the saccule in embryos and adults. However, in embryos, the saccule and utricle are known to communicate widely to provide a common endolymph space "atrium". METHODS: Using sagittal histological sections from five embryos (crown-rump length or CRL, 14-21 mm), nine early fetuses (CRL 24-35 mm) and 12 midterm and near-term fetuses (CRL 82-272 mm), we revisited the development and growth of the human ear aqueduct. RESULTS: The atrium took on a thick tube-like appearance as an antero-inferior continuation of the aqueduct, but soon divided into multiple gulfs. Most of the gulfs corresponded to the ampullae of semicircular ducts, while one gulf at the antero-medio-inferior corner corresponded to the future saccule. Notably, in eight of the 14 embryos and early fetuses, the aqueduct ended at the utricle near the primitive ampulla of the anterior (superior) or posterior semicircular duct. Conversely, an embryo of CRL 21 mm was the smallest specimen in which the aqueduct joined the gulf-like saccule. At midterm and near-term, the growing perilymph space separated the aqueduct from the utricle and appeared to push the aqueduct toward the saccule. A topographical change occurred between the embryonic superiorly located utricle and the inferiorly-located saccule to create the antero-posterior arrangement in adults. CONCLUSIONS: Consequently, the vestibular end of the aqueduct was most likely to migrate anteriorly from the utricle to the saccule at 6-8 weeks possibly due to differential growth of the endothelium. Previous reconstructions of the embryonic aqueduct might be biased by the adult morphology.

Effects of Ndufs4 Deletion on Hearing after Various Acoustic Exposures.

Mitochondrial dysfunction can cause cochlear dysfunction and accelerate noise-induced hearing loss (NIHL). NADH dehydrogenase (ubiquinone) Fe-S protein 4 (Ndufs4) is one of the subunits of mitochondrial complex I and has a role in the assembly and stabilization of complex I. However, the involvement of Ndufs4 in the pathogenesis of NIHL has not been reported. The aim of this study was to evaluate whether Ndufs4 deletion causes vulnerability to noise exposures. The wild-type (WT) and Ndufs4 knockout (KO) mice with C57BL/6J genetic background were used. Cochlear histology and hearing thresholds were assessed after noise exposure at 100 or 86 dB sound pressure level (SPL). Immunostaining showed the widespread expression of Ndufs4 in the cochlea. After noise exposure at 100 dB SPL, auditory brainstem response (ABR) threshold shifts at 4 kHz in Ndufs4 KO mice were significantly higher than that in WT mice. After noise exposure at 86 dB SPL, ABR threshold shifts, wave 1 amplitudes, and the number of synapses in the inner hair cells were not significantly different. RNA sequencing revealed the decreased expression of energy generation-related genes inNdufs4 KO mice. Ndufs4 deficiency accelerates permanent low-frequency threshold shifts after moderate noise exposure.

Possibility of additive effects by the presentation of visual information related to distractor sounds on the contra-sound effects of the N100m responses.

Auditory-evoked responses can be affected by different types of contralateral sounds or by attention modulation. The present study examined the additive effects of presenting visual information about contralateral sounds as distractions during dichotic listening tasks on the contralateral effects of N100m responses in the auditory-evoked cortex in 16 subjects (12 males and 4 females). In magnetoencephalography, a tone-burst of 500 ms duration at a frequency of 1000 Hz was played to the left ear at a level of 70 dB as a stimulus to elicit the N100m response, and a movie clip was used as a distractor stimulus under audio-only, visual-only, and audio-visual conditions. Subjects were instructed to pay attention to the left ear and press the response button each time they heard a tone-burst stimulus in their left ear. The results suggest that the presentation of visual information related to the contralateral sound, which worked as a distractor, significantly suppressed the amplitude of the N100m response compared with only the contralateral sound condition. In contrast, the presentation of visual information related to contralateral sound did not affect the latency of the N100m response. These results suggest that the integration of contralateral sounds and related movies may have resulted in a more perceptually loaded stimulus and reduced the intensity of attention to tone-bursts. Our findings suggest that selective attention and saliency mechanisms may have cross-modal effects on other modes of perception.

Tympanic membrane findings and Eustachian tube function after transtympanic plugging for the chronic patulous Eustachian tube.

OBJECTIVE: To evaluate Eustachian tube (ET) function after Kobayashi plug surgery based on the tympanic membrane (TM) findings and active opening (AO) of the ET assessed with sonotubometry. SUBJECTS AND METHODS: A retrospective survey of medical records identified 74 ears of 66 patients with patulous ET (PET) received transtympanic insertion of the Kobayashi plug. Excluding the six ears (6 patients) with abnormal preoperative TM, sixty-eight ears of 60 patients were found to have normal TM preoperatively. Among these 68 ears, there were 51 ears in which sonotubometry was performed both before and after surgery to evaluate whether the AO of the ET was positive or not. RESULTS: Out of the 68 ears with normal preoperative TM, 52 ears (76.5%) were judged successful (sum of complete relief and significant improvement). The postoperative TM was normal in 41 ears (60.3%), while 27 ears (39.7%) had abnormal TM findings postoperatively. The success rate was 75.6% (31/41) in ears with normal postoperative TM, while it was 77.8% (21/27) in ears with abnormal TM. Success in maintaining normal postoperative TM was found in 45.6% (31/68) of the total ears treated. Out of the 51 ears in which sonotubometry was performed both before and after surgery, AO was preoperatively positive in 88.2% of the ears (45/51), while it was positive in 64.7% (33/51) postoperatively. In thirty-four ears with normal TM postoperatively, AO was positive in 24 ears (70.6%), while it was positive in 9 out of 17 ears (52.9%) with abnormal postoperative TM. The success rate was 70.6% (36/51) for the 51 ears in which AO was assessed both pre- and postoperatively, and it was 66.7% (22/33) in ears with positive AO postoperatively, while it was 77.8% (14/18) in ears without AO postoperatively. The incidence of ears either having normal postoperative TM or positive AO postoperatively was 84.3% (43/51). Abnormal postoperative TM findings without effectiveness were found in 8.8% (6/68). CONCLUSION: The obstructive dysfunction of the ET is a calculated risk but did not occur in most ears after plugging with the Kobayashi plug. Therefore, routine insertion of the VT at the same time as the initial surgery is not recommended for PET cases that are adequately followed up.

Management of cholesterol granuloma due to eosinophilic otitis media using large ventilation tube.

A 46-year-old man who had been diagnosed with eosinophilic otitis media (EOM) and eosinophilic chronic rhinosinusitis was referred to our department. He suffered from bilateral earache, clogged ear sensation, and otorrhea associated with EOM. He had been treated with a myringotomy and a ventilation tube (VT) insertion. However, his symptoms did not improve, and the VT repeatedly fell out. We performed canal wall down mastoidectomy via a retro-auricular incision to remove the presumed cholesterol granuloma (CG) and a long-term VT insertion. The VT fell out repeatedly. Therefore, a large VT that Komune devised was inserted. Four months after reinsertion, there was no evidence of recurrent otorrhea or fallout of a large VT. A large VT insertion could be useful in the severe case of EOM with CG.

Long-term tumor growth and hearing after conservative management of vestibular schwannomas.

BACKGROUND: There has been no report of a detailed long-term study of hearing at defined frequencies. AIMS/OBJECTIVES: This study aimed to evaluate long-term vestibular shwannoma (VS) growth and long-term changes in hearing thresholds at defined frequencies. METHODS: We retrospectively retrieved the medical records of 67 VS patients. Cases that were followed up for more than 5 years were analyzed. RESULTS: Tumor growth was observed in 15 cases (22.4%) and 6 cases underwent gamma knife treatment (9.0%). The longest case of tumor growth and gamma knife treatment was observed 11 years after the initial diagnosis. Hearing thresholds at 500-2000 Hz was significantly different between the no growth and growth group (p < .05). Particularly at 1000 Hz, there was a significant difference between the two groups from an early time-point. CONCLUSIONS AND SIGNIFICANCE: Even a 10-year or longer follow-up of VS may show an increase in tumor growth. Moreover, hearing thresholds, particularly at 1000 kHz, may predict tumor growth when following-up VS patients.

Familial Paget's disease of bone with ocular manifestations and a novel TNFRSF11A duplication variant (72dup27).

INTRODUCTION: Paget's disease of bone (PDB) is a skeletal disorder characterized by disorganized bone remodeling due to abnormal osteoclasts. Tumor necrosis factor receptor superfamily member 11A (TNFRSF11A) gene encodes the receptor activator of nuclear factor kappa B (RANK), which has a critical role in osteoclast function. There are five types of rare PDB and related osteolytic disorders due to TNFRSF11A tandem duplication variants so far, including familial expansile osteolysis (84dup18), expansile skeletal hyperphosphatasia (84dup15), early-onset familial PDB (77dup27), juvenile PDB (87dup15), and panostotic expansile bone disease (90dup12). MATERIALS AND METHODS: We reviewed a Japanese family with PDB, and performed whole-genome sequencing to identify a causative variant. RESULTS: This family had bone symptoms, hyperphosphatasia, hearing loss, tooth loss, and ocular manifestations such as angioid streaks or early-onset glaucoma. We identified a novel duplication variant of TNFRSF11A (72dup27). Angioid streaks were recognized in Juvenile Paget's disease due to loss-of-function variants in the gene TNFRSF11B, and thought to be specific for this disease. However, the novel recognition of angioid streaks in our family raised the possibility of occurrence even in bone disorders due to TNFRSF11A duplication variants and the association of RANKL-RANK signal pathway as the pathogenesis. Glaucoma has conversely not been reported in any case of Paget's disease. It is not certain whether glaucoma is coincidental or specific for PDB with 72dup27. CONCLUSION: Our new findings might suggest a broad spectrum of phenotypes in bone disorders with TNFRSF11A duplication variants.

Auditory Attention Ability under Dichotic Dual-Task Situation in Adults with Listening Difficulties.

INTRODUCTION: Recent studies have reported poor cognition, such as attention and working memory, in adults with listening difficulties (LiD). However, they do not adequately describe the actual state of poor attention ability in adults with LiD. We examined the state of auditory attention in adults with and without LiD in tasks requiring multiple attention controls. METHODS: Twenty-one adults who had normal hearing but complained about LiD encountered during everyday life and 22 healthy controls were included. We presented a target detection task using an odd-ball format for one ear and a sentence repetition task for the other ear. In the target detection task, participants listened to the 1,000-Hz tone served as the standard stimulus, while they had to accept a 2,000-Hz tone presented as the deviant stimulus. In the sentence repetition task, short sentences were presented. The stimuli presented to them were played on a personal computer at the most comfortable level. The participants heard these stimuli through headphones. They were required to press a key for standard stimuli in the target detection task and repeat what they heard immediately in the repetition task. We compared the response accuracy for each ear task between adults with and without LiD. RESULTS: Our results showed that there were significant differences between the participant groups in the auditory dual-task under the dichotic listening situation. When examined individually, four adults with LiD had decreased scores in both the sentence repetition and target detection task, while the other nine participants showed a bias toward either task. Furthermore, the analysis of reaction time for pressing button revealed that the standard deviation of reaction time was extended in participants who scored poorly in either of the ear tasks. On the other hand, all adults without LiD were able to conduct the auditory dual-task exactly and promptly. CONCLUSION: The results suggest that adults with LiD have difficulties in appropriately allocating various cognitive abilities required for each task. We concluded that auditory attention is an important ability to conduct the auditory dual-task, and this is applicable for adults with LiD. Therefore, we believe that it is necessary to use auditory tests that require complex attentional abilities in listening, such as those required in daily life, to assess adults with LiD.

Immunohistochemical localization of d-ß-aspartic acid in congenital and acquired middle ear cholesteatoma.

Objective/Hypothesis: Middle ear cholesteatoma is characterized by abnormal growth of the keratinizing squamous epithelium of the temporal bone. d-ß-aspartic acid is the major isomer of d-aspartic acid found in elderly tissue. We assessed the immunoreactivity to k-ß-aspartic acid of congenital and acquired middle ear cholesteatomas. Study Design: Case-control studies. Material and Methods: Tissue samples were collected from 21 patients comprising 21 ears with congenital middle ear cholesteatoma and 26 patients comprising 29 ears with acquired type. Their clinical and histopathological features were investigated. We divided the middle ear cholesteatoma samples into three layers: the perimatrix, matrix, and cystic contents. The patterns of immunoreactivity to d-ß-aspartic acid expression were then assessed immunohistochemically. Results: Two patterns of immunoreactivity to d-ß-aspartic acid were detected in middle ear cholesteatoma: infiltrative and diffuse. In congenital middle ear cholesteatoma, d-ß-aspartic acid expression was observed throughout all the layers (perimatrix, matrix, and cystic contents), and immunoreactivity to d-ß-aspartic acid was dramatically strong in all layers. The expression levels of d-ß-aspartic acid to the cystic content and perimatrix were significantly higher in congenital middle ear cholesteatoma than in the acquired type. Conclusions: This study showed the expression levels of d-ß-aspartic acid in middle ear cholesteatoma to differ significantly between congenital and acquired middle ear cholesteatoma. Our results indicate that overexpression of d-ß-aspartic acid is likely to be involved in the pathogenesis of cholesteatoma, and we speculate that d-ß-aspartic acid could be a novel biomarker for, and a therapeutic target in, congenital and acquired middle ear cholesteatoma. Level of Evidence: 4.

Temporal and Directional Cue Effects on the Cocktail Party Problem for Patients With Listening Difficulties Without Clinical Hearing Loss.

OBJECTIVES: To evaluate the listening difficulty in a cocktail party environment in the sound field in order to better demonstrate patients' difficulties listening in noise, and to examine temporal and directional cue effects on the speech intelligibility in patients with listening difficulties in noise in comparison with control subjects. DESIGN: This study examined and analyzed 16 control subjects without any complaints of listening difficulties and 16 patients who had visited the outpatient clinic of the Department of Otolaryngology-Head and Neck Surgery, Tohoku University Hospital, with complaints of listening difficulties, especially in background crowded conditions, despite having relatively good hearing on routine audiograms and speech audiometry. Using five loudspeakers located in front of the subject and at 30° and 60° to the left and right from the front, word intelligibility for the target voice (female talker) presented from one of the loudspeakers in random order with four distractor voices (male talker) was assessed under the following cue conditions: (1) "no additional temporal/directional cue (only talker sex as a cue)"; (2) "fixed temporal cue without directional cue" (white noise bursts [cue sounds] were presented from the five loudspeakers just before word presentation at 500-ms intervals); (3) "directional + variable temporal cues" [cue sounds were presented from the loudspeaker where the next target word would be presented with a variable inter-stimulus interval [ISI] of 500, 1000, 1500, or 2000 ms between the cue sound and word presentation); and (4) "directional + fixed temporal cues" (cue sounds were presented from the loudspeaker where the next target word would be presented with a fixed ISI of 500 ms). RESULTS: The results indicated the following: (1) word intelligibility under distractors was significantly deteriorated in patients with listening difficulties compared with control subjects, although the clinical speech in noise test using the headphone system did not show any significant differences between the two groups; (2) word intelligibility under distractors for patients with listening difficulties was significantly improved with directional cues presented in advance; and (3) under most cue conditions, individual differences in word intelligibility among patients with listening difficulties were significantly correlated with their dichotic listening ability, which is one of the indicators used to assess auditory selective attention ability. CONCLUSIONS: The results of this study indicate the usefulness of the presentation of directional cues for speech comprehension in the cocktail party situation in patients with listening difficulties, as well as the importance of evaluating the degree of listening difficulties spatially in the cocktail party situation.

Outcome of the 'waiting until spontaneous extrusion' strategy for long-term tympanostomy tube placement in children with cleft palate.

BACKGROUND: Otitis media with effusion (OME) in children with cleft palate (CP) is known to be refractory to treatment and most of these patients undergo surgery for ventilation tube (VT) placement. OBJECTIVES: To identify the outcomes of children with CP using long-term VT with a 'waiting until spontaneous extrusion' strategy. MATERIAL AND METHODS: We retrospectively reviewed the medical records of all children with CP who visited our department from December 2016 to November 2017 and who received long-term VT placement in our department. Risk factors related to residual perforation and recurrence of OME were analyzed. RESULTS: A total of 106 children were included in this study. Our statistical analysis of 94 ears followed for more than three months after VT loss revealed that longer VT placement was associated with residual perforation, and shorter VT placement was associated with OME recurrence. Although a longer duration of VT placement was associated with an increased rate, extremely long-term VT placement was not associated with residual perforation, as expected. Half of the VTs were spontaneously extruded at 40 months after insertion. CONCLUSIONS AND SIGNIFICANCE: Long-term VT insertion using a waiting until spontaneous extrusion strategy is a potential option for children with CP.

Malignant otitis externa presenting cerebral infarction from pseudoaneurysm: A case report and a review of the literature.

Chronic renal failure and diabetes mellitus could also be risk factors of pseudoaneurysm of the internal carotid artery (ICA) due to malignant otitis externa (MOE). Although pseudoaneurysm of the ICA is a rarely encountered disease, it should always be taken into consideration when treating patients of MOE.

Patulous Eustachian Tube Patients With Oculopharyngeal Muscular Dystrophy.

OBJECTIVES: To describe cases of patulous Eustachian tube (PET) or patent ET conditions in oculopharyngeal muscular dystrophy (OPMD). PATIENTS: Four cases of PET or patent ET conditions with OPMD. MAIN OUTCOME MEASURES: Clinical case records, objective ET function tests (tubo-tympano-aerodynamic graphy and sonotubometry), and swallowing function (videoendoscopic examination and Food Intake Level Scale) were analyzed. RESULTS: Two cases of definite PET, one case of possible PET, and one case lacking aural symptoms with findings of patent ET. All patients have ptosis, and three cases have dysphagia. Body mass index indicated that three cases were underweight. Magnetic resonance imaging in case 4 showed atrophy and fat replacement of palatine and masticatory muscles. CONCLUSIONS: It is important to consider PET or patent ET conditions when OPMD patients describe aural symptoms.

Enlargement of Eustachian Tube Lumen in Patulous Eustachian Tube Patients Compared to that by Aging.

OBJECTIVE: To evaluate the Eustachian tube (ET) dimensions in patulous ET (PET) patients compared with that by aging using sitting 3D computed tomography (CT). STUDY DESIGN: Retrospective study. SETTING: Tertiary referral center. SUBJECTS AND METHODS: A retrospective survey of medical records in Sen-En Rifu Hospital identified 105 ears of 76 PET patients and 65 ears of 34 patients without ET dysfunction findings (non-PET). Subjects in both PET and non-PET groups were then divided into two age groups. Groups A and C defined as non-PET and PET subjects respectively, who were under the age of 60 years, while Groups B and D defined of non-PET and PET subjects respectively, who were 60 years and above. 3D CT (Accuitomo; Morita, Kyoto, Japan) was performed on all subjects in the sitting position. The ET lumen from the ET pharyngeal orifice to 15 mm was analyzed. RESULTS: No significant difference in the ET lumen near the pharyngeal orifice was found between Group A and C; however, there was a significant difference in the ET at points lateral to the pharyngeal orifice. The ET lumen was significantly larger at the site close to the pharyngeal orifice in Group B as compared to that of Group A. For the two groups of PET classified according to the size of the pharyngeal orifice and area close to the isthmus, age was only significantly different between two groups of pharyngeal orifice. On the contrary, sonotumometry and Ohta method were significantly different between the two groups of the area close to the isthmus. CONCLUSION: The lumen of the ET is enlarged in both PET and aging. However, the responsible site was found to be different. While the enlargement of the ET lumen in 60 years and above subjects without PET mainly occurred near the pharyngeal orifice of the ET, it was near the isthmus in under 60 years PET patients. Further study of possible clinical implications of these findings as well as treatment strategy are required.

Hearing Outcomes of Transmastoid Plugging for Superior Canal Dehiscence Syndrome by Underwater Endoscopic Surgery: With Special Reference to Transient Bone Conduction Increase in Early Postoperative Period.

OBJECTIVE: To investigate the influence on hearing of transmastoid plugging of the superior semicircular canal accompanied with membranous superior canal transection by underwater endoscopic ear surgery (UWEES) for the superior semicircular canal dehiscence syndrome. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral center. PATIENTS: Seven patients underwent plugging with membranous superior canal transection with UWEES from 2017 to 2019. INTERVENTION: Bone conduction (BC) thresholds (250, 500, 1000, 2000, 4000 Hz) were repetitively examined in early postoperative period. MAIN OUTCOME MEASURES: Subjective symptoms and pure-tone audiometry. RESULTS: Transient BC threshold increase was detected in all cases in early postoperative period and hearing levels were ameliorated in 1 to 2 months. The mean maximum BC threshold elevations (dB) during the early postoperative period (within 1 mo) and the postoperative stable hearing period (after 2 mo) were 18.6 and 2.9 at 250 Hz, 24.3 and 8.6 at 500 Hz, 26.4 and 8.6 at 1000 Hz, 28.6 and 7.1 at 2000 Hz, and 30.0 and 0.8 (except for scale-out cases) at 4000 Hz. respectively. The mean maximum BC thresholds in the early period were significantly elevated compared with those in the stable period at each frequency (p < 0.01). CONCLUSIONS: Hearing outcomes of transmastoid plugging with transection of the membranous superior canal by using UWEES were found favorable in a long-term follow-up. However, it caused transient reversible hearing loss in all cases. The BC increase in early postoperative period may not cause permanent hearing loss but improvement for the surgical technique may still be necessary.

Middle ear adenoma with facial palsy: A case report and a review of the literature.

A 52-year-old man presented to our emergency department with an acute onset of right-sided facial nerve (FN) palsy of House-Brackmann grade V. Electroneurography (ENoG) was conducted with no response at the right FN, as compared with the left FN (0%). We performed a biopsy of the right middle ear mass and histological studies showed the tumor to be neuroendocrine tumors (NET) of the middle ear. We resected the tumor with canal wall down mastoidectomy and reconstructed the posterior meatal wall with soft tissue. Three months after surgery, the FN paralysis had improved with House-Brackmann grade II. We reviewed cases of NET with FN palsy, and nine patients, including our case, have been reported. Our case is the first report of ENoG for the description of FN palsy due to NET. Although the ENoG value was 0%, it was remarkably improved by surgery. The other cases of NET patients with FN palsy also recovered FN function after surgery. These results suggest that it is recommended to perform the total resection of the tumor to improve the FN function.