Journal of Otology最新文献

Background and purpose

Cerebellar ataxia, neuropathy, and vestibular areflexia syndrome (CANVAS) is a neurodegenerative disease of the cerebellum. The disease progression is slow, with up to 25% of people diagnosed needing to use a wheelchair after 15 years from diagnosis. Vestibular symptoms arise from centrally-mediated ocular movement degradation and the reduced vestibular-ocular reflex functioning bilaterally. To date, no report has shown an improvement in VOR gain or gait outcome measures in someone with CANVAS after a course of vestibular physical therapy.

Case description

A 65-year-old male, Patient X, first noticed symptoms in his fourth decade of life and was diagnosed with (CANVAS) in his seventh decade. Patient X reported numbness and tingling in his hands and feet, decreased ability to perform daily activities, and several falls.

Intervention

Patient X completed a four-month course of vestibular physical therapy, including vestibular ocular reflex exercises, balance training, gait training, and the VestAid application for eye gaze compliance monitoring. The Vestaid application uses eyes and facial recognition software to record the percentage of time that the patient kept their eyes on the target.

Outcomes

After vestibular therapy, Patient X had a clinically meaningful improvement in gait speed: from 1.02 m/s to 1.13 m/s and in the Functional Gait Assessment from 20/30 to 27/30. Patient X's eye gaze compliance improved from a median of 43% (range 25–68%) to a median of 67% (58–83%).

Discussion

This case study demonstrates that vestibular rehabilitation improved eye gaze compliance and functional outcomes in a person living with CANVAS.

Nodular fasciitis is a benign reactive soft tissue tumor arising from fibroblasts and myofibroblasts. Its incidence is low and misdiagnosis is frequent especially for malignant lesions. This can lead to inappropriate and unnecessary invasive treatment. Nodular fasciitis of the external auditory canal is extremely rare. So far, around fifteen cases have been reported. We present here the case of a 90-year-old patient with nodular fasciitis of the right external auditory canal. The lesion extends anteriorly for 6.5 cm and reaches the posterior wall of the maxillary sinus. To our knowledge, this is the first case in the literature of an external auditory canal nodular fasciitis presenting as an inflammatory ear polyp with such a wide extension.

Objective

The purpose of the study was to evaluate listening effort in adults who experience varied annoyance towards noise.

Materials and methods

Fifty native Kannada-speaking adults aged 41–68 years participated. We evaluated the participant's acceptable noise level while listening to speech. Further, a sentence-final word-identification and recall test at 0 dB SNR (less favorable condition) and 4 dB SNR (relatively favorable condition) was used to assess listening effort. The repeat and recall scores were obtained for each condition.

Results

The regression model revealed that the listening effort increased by 0.6% at 0 dB SNR and by 0.5% at 4 dB SNR with every one-year advancement in age. Listening effort increased by 0.9% at 0 dB SNR and by 0.7% at 4 dB SNR with every one dB change in the value of Acceptable Noise Level (ANL). At 0 dB SNR and 4 dB SNR, a moderate and mild negative correlation was noted respectively between listening effort and annoyance towards noise when the factor age was controlled.

Conclusion

Listening effort increases with age, and its effect is more in less favorable than in relatively favorable conditions. However, if the annoyance towards noise was controlled, the impact of age on listening effort was reduced. Listening effort correlated with the level of annoyance once the age effect was controlled. Furthermore, the listening effort was predicted from the ANL to a moderate degree.

Purpose

To construct a symptoms-based prediction tool to assess the likelihood of superior canal dehiscence (SSCD) on high-resolution CT.

Materials and methods

Mathematical modeling was employed to predict radiologic evidence of SSCD at a tertiary neurotology referral center.

Results

A total of 168 patients were included, of which 118 had imaging-confirmed SSCD. On univariate analysis significant predictors of SSCD presence were: sound/pressure-induced vertigo (p = 0.006), disequilibrium (p = 0.008), hyperacusis (p = 0.008), and autophony (p = 0.034). Multivariate analysis enabled a 14-point symptom-weighted tool to be developed, wherein a score of ≥6 raised the suspicion of SSCD (≥70% likelihood of being present), R² = 0.853.

Conclusions

The likelihood of SSCD on CT scan can be determined with a high degree of certainty based on symptoms recorded at presentation. Using the evidenced-based diagnostic tool validated herein, a score ≥6 with any symptom combination justifies ordering a CT scan.

Objective

To investigate the potential influence of anatomical variation in the anterior inferior cerebellar artery (AICA) on the occurrence and severity of idiopathic sudden sensorineural hearing loss (ISSNHL).

Methods

Ninety ISSNHL patients were enrolled. The anatomical location of the AICA was exhibited using high-resolution magnetic resonance imaging (MRI), and the various AICA types classified by previously reported Chavda and Gorrie methods were analyzed. The severity of hearing loss in the ipsilateral ear among different AICA types was compared.

Results

Approximately 85.6% of subjects had unilateral ISSNHL (uISSNHL), and the others had bilateral ISSNHL (bISSNHL). In the uISSNHL group, the ratios of different AICA types were similar between the ipsilateral and contralateral ears. The ratios of the different AICA types in the bISSNHL group were similar to those in the uISSNHL group. In the uISSNHL group, pure tone audiometry (PTA) thresholds at 2 kHz, 4 kHz and 8 kHz of patients with Chavda type II AICA were higher than those of patients with Chavda type I and type III, with a significant difference at 4 kHz between type I and type II. There was a tendency of the PTA threshold in patients with Chavda type II or Gorrie type C to gradually increase from low to high frequency zones.

Conclusion

When the AICA enters the IAC (Chavda type II) or crosses between the 7th and 8th cranial nerves (Gorrie type C), the severity and frequency of hearing impairment in ISSNHL but not the occurrence of ISSNHL will be affected.

The role of intercalatus nucleus of Staderini (INS), the most caudal of the perihypoglossal nuclei, is much debated. Last research seems to suggest that this nucleus plays a role as a vertical eyes movements integrator. The few clinical reports present in the literature that describe isolated lesions of the INS have described patients presenting in acute with up-beating vertical spontaneous nystagmus. Isolated acute lesion of INS is, in fact, much rare, and, without other neurological signs, is exceptional. We present a case of acute isolated vertigo with no other neurological signs or symptoms, due to INS ischemia provoked by vertebral artery stenosis. The patient presented with spontaneous vertical up-beating nystagmus that showed at videonystagmographic recording, a clear exponential decay of angular slow-phase velocity, that is considered a typical sign of neural integrator impairment. This case seems to represent a further confirm that INS is part, as a vertical-to-position neural integrator, of the neural circuit controlling the vertical eyes movements.

Nodular fasciitis is a benign reactive soft tissue tumor arising from fibroblasts and myofibroblasts. Its incidence is low and misdiagnosis is frequent especially for malignant lesions. This can lead to inappropriate and unnecessary invasive treatment. Nodular fasciitis of the external auditory canal is extremely rare. So far, around fifteen cases have been reported. We present here the case of a 90-year-old patient with nodular fasciitis of the right external auditory canal. The lesion extends anteriorly for 6.5 cm and reaches the posterior wall of the maxillary sinus. To our knowledge, this is the first case in the literature of an external auditory canal nodular fasciitis presenting as an inflammatory ear polyp with such a wide extension.

Safe and efficient drug delivery to the inner ear has always been the focus of prevention and treatment of sensorineural deafness. The rapid development of nanodrug delivery systems based on hydrogel has provided a new opportunity. Among them, thermo-sensitive hydrogels promote the development of new dosage form for intratympanic injection. This smart biomaterial could transform to semisolid phase when the temperature increased. Thermo-sensitive hydrogel nanodrug delivery system is expected to achieve safe, efficient, and sustained inner ear drug administration. This article introduces the key techniques and the latest progress in this field.

Objective

The purpose of the study was to evaluate listening effort in adults who experience varied annoyance towards noise.

Materials and methods

Fifty native Kannada-speaking adults aged 41–68 years participated. We evaluated the participant's acceptable noise level while listening to speech. Further, a sentence-final word-identification and recall test at 0 dB SNR (less favorable condition) and 4 dB SNR (relatively favorable condition) was used to assess listening effort. The repeat and recall scores were obtained for each condition.

Results

The regression model revealed that the listening effort increased by 0.6% at 0 dB SNR and by 0.5% at 4 dB SNR with every one-year advancement in age. Listening effort increased by 0.9% at 0 dB SNR and by 0.7% at 4 dB SNR with every one dB change in the value of Acceptable Noise Level (ANL). At 0 dB SNR and 4 dB SNR, a moderate and mild negative correlation was noted respectively between listening effort and annoyance towards noise when the factor age was controlled.

Conclusion

Listening effort increases with age, and its effect is more in less favorable than in relatively favorable conditions. However, if the annoyance towards noise was controlled, the impact of age on listening effort was reduced. Listening effort correlated with the level of annoyance once the age effect was controlled. Furthermore, the listening effort was predicted from the ANL to a moderate degree.

Purpose

To construct a symptoms-based prediction tool to assess the likelihood of superior canal dehiscence (SSCD) on high-resolution CT.

Materials and methods

Mathematical modeling was employed to predict radiologic evidence of SSCD at a tertiary neurotology referral center.

Results

A total of 168 patients were included, of which 118 had imaging-confirmed SSCD. On univariate analysis significant predictors of SSCD presence were: sound/pressure-induced vertigo (p = 0.006), disequilibrium (p = 0.008), hyperacusis (p = 0.008), and autophony (p = 0.034). Multivariate analysis enabled a 14-point symptom-weighted tool to be developed, wherein a score of ≥6 raised the suspicion of SSCD (≥70% likelihood of being present), R² = 0.853.

Conclusions

The likelihood of SSCD on CT scan can be determined with a high degree of certainty based on symptoms recorded at presentation. Using the evidenced-based diagnostic tool validated herein, a score ≥6 with any symptom combination justifies ordering a CT scan.