This study aimed to determine if pre-operative radiological scoring can reliably predict intra-operative difficulty and final cochlear electrode position in patients with advanced otosclerosis.

A retrospective cohort study of advanced otosclerosis patients who underwent cochlear implantation (n = 48, 52 ears) was compared with a larger cohort of post-lingually deaf adult patients (n = 1414) with bilateral hearing loss and normal cochlear anatomy. Pre-operative imaging for advanced otosclerosis patients and final electrode position were scored and correlated with intra-operative difficulty and speech outcomes.

Advanced otosclerosis patients benefit significantly from cochlear implantation. Mean duration of deafness was longer in the advanced otosclerosis group (19.5 vs 14.3 years; p < 0.05).

Anatomical changes in advanced otosclerosis can result in increased difficulty of surgery. Evidence of pre-operative cochlear luminal changes was associated with intra-operative difficult insertion and final non-scala tympani position. Nearly all electrodes implanted in the advanced otosclerosis cohort were peri-modiolar. No reports of facial nerve stimulation were observed.

Children with single-sided deafness often receive inconsistent clinical recommendations because there is currently no clear best practice in paediatric single-sided deafness. This systematic review of the literature aimed to compare commonly used treatments and attempted to support the use of a particular treatment modality.

This was a comprehensive literature review from 1 January 2000 to 22 February 2022; the study compared the outcomes of bone conduction devices and cochlear implantation in paediatric patients with single-sided deafness.

Fifteen studies consisting of 202 patients were examined. Variables including speech reception in quiet and noise, as well as quality of life measures were compared. Both cochlear implantation and bone-anchored hearing aids demonstrated benefits in sound perception. Quality of life measures improved with both modalities.

Although both bone-anchored hearing aids and cochlear implantation appear to provide significant improvements, additional research with more direct comparisons is needed to provide more decisive results.

This study aimed to determine the probability of hearing recovery in patients with idiopathic sudden sensorineural hearing loss following salvage intratympanic steroids

A retrospective review of all patients receiving salvage intratympanic steroid injections for idiopathic sudden sensorineural hearing loss was performed (January 2014 to December 2019). Twenty-two patients were identified, of whom 15 met inclusion criteria. Pre- and post-treatment audiograms were compared with the unaffected ear. Hearing recovery was categorised based on American Academy of Otolaryngology Head and Neck Surgery criteria.

Only 1 patient out of 15 (6.7 per cent) made a partial recovery, and the remainder were non-responders. The median duration of time between symptom onset and first salvage intratympanic steroid treatment was 52 days (range, 14–81 days). No adverse reactions were observed.

‘Real world’ patients with idiopathic sudden sensorineural hearing loss present differently to those in the literature. Sudden sensorineural hearing loss should be diagnosed with care and intratympanic steroid injections initiated early if considered appropriate. Patients should make an informed decision on treatment based on prognostic factors and local success rates.

Sensorineural hearing loss following spinal anaesthesia, epidural anaesthesia or lumbar puncture is a rare phenomenon that is thought to occur when reduced cerebrospinal fluid pressure is transmitted to the inner ear through an enlarged cochlear aqueduct.

This study presents two cases of sensorineural hearing loss following spinal anaesthesia for caesarean section as well as presenting results of a systemic review of the available literature using the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. Full-text articles from Medline, PubMed and Embase were used, as well as associated reference lists. Risk factors associated with poorer outcomes and an approach to management of this rare condition are also discussed.

Twenty-one cases were identified. The literature was systematically reviewed showing presentations, investigations performed, treatments offered and outcomes.

Sensorineural hearing loss following spinal anaesthesia, epidural anaesthesia or lumbar puncture is a rare occurrence that requires a high degree of clinical suspicion and prompt investigation and treatment.

To determine the effect of cochlear dimensions on cochlear implant selection in cochlear hypoplasia patients.

Temporal bone computed tomography images of 36 patients diagnosed with cochlear hypoplasia between 2010 and 2016 were retrospectively reviewed and compared with those of 40 controls without sensorineural hearing loss.

Basal turn length and mid-modiolar height were significantly lower in the cochlear hypoplasia patients with subtypes I, II and III than in the control group (p < 0.001). Mid-scalar length was significantly shorter in subtype I–III patients as compared with the control group (p < 0.001). In addition, cochlear canal length (measured along the lateral wall) was significantly shorter in subtype I–IV patients than in the control group (subtypes I–III, p < 0.001; subtype IV, p = 0.002)

Cochlear hypoplasia should be considered if basal turn length is less than 7.5 mm and mid-modiolar height is less than 3.42 mm. The cochlear implant should be selected according to cochlear hypoplasia subgroup. It is critically important to differentiate subtype II from incomplete partition type I and subtype III from a normal cochlea, to ensure the most appropriate implant electrode selection so as to optimise cochlear implantation outcomes.

This study aimed to evaluate hearing outcomes and device safety in a large, single-surgeon experience with the totally implantable active middle-ear implants.

This was a retrospective case series review of 116 patients with moderate-to-severe sensorineural hearing loss undergoing implantation of active middle-ear implants.

Mean baseline unaided pure tone average improved from 57.6 dB before surgery to 34.1 dB post-operatively, signifying a mean gain in pure tone average of 23.5 dB (p = 0.0002). Phonetically balanced maximum word recognition score improved slightly from 70.5 per cent to 75.8 per cent (p = 0.416), and word recognition score at a hearing level of 50 dB values increased substantially from 14.4 per cent to 70.4 per cent (p < 0.0001). Both revision and explant rates were low and dropped with increasing surgeon experience over time.

This study showed excellent post-operative hearing results with active middle-ear implants with regard to pure tone average and word recognition score at a hearing level of 50 db. Complication rates in this case series were significantly lower with increasing experience of the surgeon. Active middle-ear implants should be considered in appropriate patients with moderate-to-severe sensorineural hearing loss who have struggled with conventional amplification and are good surgical candidates.

To determine the prevalence of cochlear nerve anomalies on magnetic resonance imaging in patients with unilateral or bilateral sensorineural hearing loss.

A retrospective case series was conducted at a tertiary referral centre. The inclusion criteria were paediatric patients with bilateral or unilateral sensorineural hearing loss, investigated with magnetic resonance imaging. The primary outcome measure was the rate of cochlear nerve hypoplasia or aplasia.

Of the 72 patients with unilateral sensorineural hearing loss, 39 per cent (28 cases) had absent or hypoplastic cochlear nerves on the affected side. Fifteen per cent (11 cases) had other abnormal findings on magnetic resonance imaging. Eighty-four patients had bilateral sensorineural hearing loss, of which cochlear nerve hypoplasia or aplasia was identified only in 5 per cent (four cases). Other abnormal findings were identified in 14 per cent (12 cases).

Paediatric patients with unilateral sensorineural hearing loss are more likely to have cochlear nerve anomalies than those patients with bilateral sensorineural hearing loss. This has important implications regarding cochlear implantation for patients with single-sided deafness.

This study aimed to evaluate the association between cochlear nerve canal dimensions and semicircular canal abnormalities and to determine the distribution of bony labyrinth anomalies in patients with cochlear nerve canal stenosis.

This was a retrospective study in which high-resolution computed tomography images of paediatric patients with severe-to-profound sensorineural hearing loss were reviewed. A cochlear nerve canal diameter of 1.5 mm or less in the axial plane was classified as stenotic. Semicircular canals and other bony labyrinth morphology and abnormality were evaluated.

Cochlear nerve canal stenosis was detected in 65 out of 265 ears (24 per cent). Of the 65 ears, 17 ears had abnormal semicircular canals (26 per cent). Significant correlation was demonstrated between cochlear nerve canal stenosis and semicircular canal abnormalities (p < 0.01). Incomplete partition type II was the most common accompanying abnormality of cochlear nerve canal stenosis (15 out of 65, 23 per cent).

Cochlear nerve canal stenosis is statistically associated with semicircular canal abnormalities. Whenever a cochlear nerve canal stenosis is present in a patient with sensorineural hearing loss, the semicircular canal should be scrutinised for presence of abnormalities.

One hundred years ago, millions of British and Allied troops were fighting in the trenches of the Great War. With a tenth of soldiers losing their lives, hearing loss seemed a low priority; however, vast numbers of troops sustained significant hearing loss.

A review was conducted of literature published between 1914 and 1925.

Soldiers were exposed to up to 185 dB of sustained noise from new, high-energy weapons, which caused ‘labyrinthine concussion’. Traumatic injuries, non-organic hearing loss and malingering were also common. One source estimated that 2.4 per cent of the army was disabled by hearing loss. However, many British doctors viewed this ‘soldier's deafness’ as a temporary affliction, resulting in soldiers being labelled as malingerers or ‘hysterical’.

Today, one can recognise that a scant evidence base and misconceptions influenced the mismanagement of hearing loss by otolaryngologists in World War I. However, noise-induced hearing loss is still very much a feature of armed conflict.

To evaluate the presence of endolymphatic hydrops in patients with immune-mediated inner-ear disease.

The presence of endolymphatic hydrops was prospectively evaluated in 17 patients clinically diagnosed with secondary (n = 5) or primary (n = 12) immune-mediated inner-ear disease, who attended the ENT department of a tertiary care centre for evaluation or treatment over the previous year. All patients underwent magnetic resonance imaging of the temporal bone.

Intratympanic gadolinium three-dimensional magnetic resonance imaging diagnosed hydrops in 11 of 12 patients with primary immune-mediated inner-ear disease (92 per cent). Of these, seven patients (64 per cent) presented only cochlear (n = 5) or predominantly cochlear (n = 2) hydrops. A positive magnetic resonance imaging result was observed in only one of five patients with secondary immune-mediated inner-ear disease (20 per cent).

This study confirms the presence of endolymphatic hydrops in immune-mediated inner-ear disease patients. The virtual absence of hydrops in patients with secondary immune-mediated inner-ear disease is remarkable, although firm conclusions cannot be drawn; this should be explored in a multicentre study with a larger sample of patients. A different immune reaction without development of endolymphatic hydrops should not be ruled out in secondary immune-mediated inner-ear disease patients.

Recent studies found that mobile phone users had a significantly greater risk of having elevated thresholds in speech frequencies. This study investigated the correlation between the laterality of sudden sensorineural hearing loss, handedness and the preferred ear for mobile phone use.

The study included all patients who presented with sudden sensorineural hearing loss to the Department of Otolaryngology – Head and Neck Surgery in our tertiary referral medical centre between 2014 and 2016. Patients were asked to indicate their dominant hand and preferred ear for mobile phone use.

The study comprised 160 patients. No correlation was found between the dominant hand or preferred ear for mobile phone use and the side of sudden sensorineural hearing loss. There was no correlation between the side of the sudden sensorineural hearing loss (preferable or non-preferable for mobile phone use) and audiometric characteristics.

No correlation was found between the laterality of ears used for mobile phone and sudden sensorineural hearing loss.

The bony cochlear nerve canal is the space between the fundus of the internal auditory canal and the base of the cochlear modiolus that carries cochlear nerve fibres. This study aimed to determine the distribution of bony labyrinth anomalies and cochlear nerve anomalies in patients with bony cochlear nerve canal and internal auditory canal atresia and stenosis, and then to compare the diameter of the bony cochlear nerve canal and internal auditory canal with cochlear nerve status.

The study included 38 sensorineural hearing loss patients (59 ears) in whom the bony cochlear nerve canal diameter at the mid-modiolus was 1.5 mm or less. Atretic and stenotic bony cochlear nerve canals were examined separately, and internal auditory canals with a mid-point diameter of less than 2 mm were considered stenotic. Temporal bone computed tomography and magnetic resonance imaging scans were reviewed to determine cochlear nerve status.

Cochlear hypoplasia was noted in 44 out of 59 ears (75 per cent) with a bony cochlear nerve canal diameter at the mid-modiolus of 1.5 mm or less. Approximately 33 per cent of ears with bony cochlear nerve canal stenosis also had a stenotic internal auditory canal and 84 per cent had a hypoplastic or aplastic cochlear nerve. All patients with bony cochlear nerve canal atresia had cochlear nerve deficiency. The cochlear nerve was hypoplastic or aplastic when the diameter of the bony cochlear nerve canal was less than 1.5 mm and the diameter of the internal auditory canal was less than 2 mm.

The cochlear nerve may be aplastic or hypoplastic even if temporal bone computed tomography findings indicate a normal cochlea. If possible, patients scheduled to receive a cochlear implant should undergo both computed tomography and magnetic resonance imaging of the temporal bone. The bony cochlear nerve canal and internal auditory canal are complementary structures, and both should be assessed to determine cochlear nerve status.

This study aimed to evaluate the presence of the N3 potential (acoustically evoked short latency negative response) in profound sensorineural hearing loss, its association with the cervical vestibular evoked myogenic potential and the relationship between both potentials and loss of auditory function.

Otological examinations of 66 ears from 50 patients aged from 4 to 36 years were performed, and the vestibular evoked myogenic potential and auditory brainstem response were measured.

The N3 potential was recorded in 36 out of 66 ears (55 per cent) and a vestibular evoked myogenic potential was recorded in 34 (52 per cent). The N3 potential was recorded in 23 out of 34 ears (68 per cent) with a vestibular evoked myogenic potential response and absent in 19 out of 32 ears (59 per cent) without a vestibular evoked myogenic potential response. The presence of an N3 potential was significantly associated with a vestibular evoked myogenic potential response (p = 0.028), but there was no significant difference in the latency or amplitude of the N3 potential in either the presence or absence of a vestibular evoked myogenic potential.

The presence of an N3 potential in profound sensorineural hearing loss with good or poor vestibular function can be explained by the contribution of the efferent cochlear pathway through olivocochlear fibres that join the inferior vestibular nerve. This theory is supported by its early latency and reversed polarity, which is masked in normal hearing by auditory brainstem response waves.

Sudden sensorineural hearing loss in divers may be caused by either inner-ear barotrauma or inner-ear decompression sickness. There is no consensus on the best treatment option. This study aimed to evaluate the therapeutic value of hyperbaric oxygen therapy for sudden sensorineural hearing loss in divers.

A literature review and three cases of divers with sudden sensorineural hearing loss treated with hyperbaric oxygen therapy are presented.

Hyperbaric oxygen therapy resulted in hearing improvement in 80 per cent of patients: 39 per cent had hearing improvement and 41 per cent had full recovery.

Hyperbaric oxygen therapy improved hearing in divers with sudden sensorineural hearing loss.

Sensorineural hearing loss is a recognised complication of cryptococcal meningitis. The mechanism of hearing loss in patients with cryptococcal meningitis is different from that in bacterial meningitis.

An immune-competent man with cryptococcal meningitis presented with sudden onset, bilateral, severe to profound sensorineural hearing loss and vestibular dysfunction. He was initially evaluated for cochlear implantation. However, he had a significant recovery; he no longer required surgery and was able to cope without a hearing aid.

Typically, cochlear implantation is performed with some urgency in patients with hearing loss post-bacterial meningitis, because of the risk of labyrinthitis ossificans. However, this process has not been described in patients with cryptococcal meningitis. Furthermore, patients with hearing loss associated with cryptococcal meningitis have shown varying degrees of reversibility. In this case report, hearing loss from cryptococcal meningitis is compared with that from bacterial meningitis, and the need for cochlear implantation in patients with cryptococcal meningitis is discussed.

To evaluate a small cohort of patients who presented with symptoms and signs consistent with acute infective sensorineural hearing loss who were treated with intratympanic steroids.

Seven patients received a 7-day course of oral antibiotics and oral prednisolone followed by 3 intratympanic injections of methylprednisolone and 1 week of topical dexamethasone drops.

Hearing improved in 57 per cent of patients (four out of seven). The mean improvement in this group was 24 dB (range, 10–52 dB). The magnitude of the sensorineural hearing loss at presentation was less in those who responded to intratympanic steroid therapy than in non-responders (mean pure tone average of 30 dB versus 65 dB pre-intratympanic steroids, and 14 dB versus 83 dB post-intratympanic steroids, respectively).

The results of our study suggest that intratympanic steroids provide a valuable contribution to the treatment of acute infective sensorineural hearing loss and may provide additional benefit by virtue of a concentrated local steroid effect in patients who do not respond to antibiotics.

Unilateral sudden sensorineural hearing loss due to an infarct in the vertebrobasilar system has been widely reported. Most patients have a background of traditional coronary risk factors related to these cerebrovascular episodes.

A 32-year-old male, a regular user of anabolic steroids, presented to the emergency department with unilateral sensorineural hearing loss and symptoms suggestive of an infarct of the anterior inferior cerebellar artery but in the absence of risk factors for ischaemic stroke.

Magnetic resonance imaging confirmed the presence of infarction in the region supplied by the anterior inferior cerebellar artery. Polycythaemia was found on haematological analysis, which we believe was secondary to the use of anabolic steroids. The patient was commenced on aspirin as per the stroke management protocol. There was resolution of neurological symptomatology six weeks after the episode, but no improvement in hearing.

To our knowledge, this is the first case report of unilateral sensorineural hearing loss secondary to the use of anabolic steroids causing polycythaemia. This cause should be considered in the differential diagnosis of patients presenting with sensorineural hearing loss, especially in young males, when no other risk factors can be identified.

Although other blood dyscrasias are known to cause sudden sensorineural hearing loss, macrocytosis has not previously been implicated in the absence of another causative agent.

We present a case of bilateral sequential sudden sensorineural hearing loss in a patient with significant macrocytosis (mean corpuscular volume at presentation 124 fl) secondary to alcohol-induced liver dysfunction.

A possible pathophysiological mechanism linking macrocytosis and sudden sensorineural hearing loss was identified, suggesting areas for further investigation.

To study the possible damage to the vestibular system in patients with post-mumps sensorineural hearing loss.

Nineteen patients with recent mumps infection participated in the study. All patients had unilateral profound sensorineural hearing loss or total hearing loss. Patients were subjected to video-nystagmography and vestibular-evoked myogenic potential testing.

Eight patients (42.1 per cent) had normal video-nystagmography results and intact vestibular-evoked myogenic potentials on both sides, whereas the other 11 patients (57.9 per cent) had vestibular lesions in the form of marked canal weakness and absent vestibular-evoked myogenic potential responses on the same side as hearing loss. The overall findings indicated a peripheral site for the lesions.

The majority of patients with post-mumps sensorineural hearing loss had peripheral vestibular pathology in the same ear as hearing loss. Further research should be directed to saving the inner ear following mumps infection.

To report magnetic resonance imaging findings in a patient with an SLC26A4 gene mutation who had low-frequency sensorineural hearing loss.

A 13-year-old girl had bilateral and symmetric low-frequency sensorineural hearing loss. Upon genetic testing, a heterozygous c.1105A > G (p.K369E) mutation of the SLC26A4 gene was detected. Mild endolymphatic hydrops in the right cochlea and marked endolymphatic hydrops in the left vestibulum were seen by magnetic resonance imaging 4 hours after an intravenous gadolinium injection.

This is the first reported case of a patient with the SLC26A4 gene mutation c.1105A > G (p.K369E) who had low-frequency sensorineural hearing loss. Co-occurrence of cochlear and vestibular endolymphatic hydrops suggests an association with that pathology.