Ms Melissa Babbage Senior Audiologist Clinic Manager Dilworth Hearing 14:00-14:55 WS #30: Sudden Sensorineural Hearing Loss and Management of Single Sided Deafness 15:05-16:00 WS #40: Sudden Sensorineural Hearing Loss and Management of Single Sided Deafness (Repeated)
Sudden Sensorineural Hearing Loss (SSHL) and Management of Single Sided Deafness South GP CME August 2018 Presented by Melissa Babbage, PhD, MNZAS CCC
Dilworth Hearing Services Hearing Tests for all ages Hearing Aid Fitting & Adjustments we are not owned by a manufacturer we fit what is best for the patient Assistive Listening Devices Cochlear Implants Tinnitus Assessment and Management Auditory Processing Assessments Hearing / Ear Protection Ear Nurses for wax removal
Goals: To be able to identify SSHL To understand the urgency for prompt treatment To update your knowledge of treatment for SSHL To develop knowledge of rehabilitation options for patients with single-sided deafness
What is SSHL? SSHL is the loss of hearing which occurs suddenly or over a few days (72 hours). This rapid loss involves the inner ear or nerve of hearing. Hearing loss can range from a mild impairment to a total loss of hearing. It usually affects one ear although hearing loss can be bilateral in around 1 2 % of cases. It should be considered a MEDICAL EMERGENCY requiring immediate recognition and attention.
Epidemiology It is estimated that SSHL affects 5 to 20 per 100,000 population Males and females are equally affected SSHL can occur at any age, but is more common in patients in their 40s, 50s, and 60s
What causes SSHL? Many possible causes, including: Bacterial or viral infection Vascular compromise Inflammation and autoimmune disease Vestibular schwannoma Trauma Ménière s disease Demyelinating disease Ototoxic drugs However, 85-90% of cases of SSHL are idiopathic.
Typical presentation of SSHL Loss of hearing in one ear Voices sound muffled on that side compared to the unaffected side Sounds seem to echo or be distorted on one side Sudden onset of tinnitus in one ear Pressure or blocked feeling in one ear A loss of balance or vertigo May notice a pop in the ear before hearing decreases
Prompt diagnosis - do not wait! Clear pathway for diagnosis and management
Management of SSHL Step 1: A history and careful physical examination are important to rule out more severe pathologies such as vascular events and malignant diseases. Exclude occluding wax or middle ear pathology (although this does not exclude a SSHL). Tuning fork tests can help differentiate between a sensorineural hearing loss or conductive hearing loss.
Management of SSHL Step 2: Urgently refer to audiology for a full diagnostic hearing test Confirm hearing loss Distinguish between conductive and sensorineural loss Determine degree of hearing impairment Establish baseline pre-treatment
What might you see on a diagnostic audiogram? This patient has partial hearing loss in both ears, much worse in the left ear where the overlay of sudden loss has occurred. This patient has total loss (dead ear) in their left ear, accompanied by no useful hearing for speech
What might you see on a diagnostic audiogram? This patient reported tinnitus, but did not perceive any change in hearing.
Management of SSHL Step 3: If SSHL is confirmed, the patient will be referred back to the GP with a recommendation to start treatment with oral corticosteroids. An urgent referral to Otolaryngology (ORL) for imaging is required. Regardless of whether the hearing returns, imaging is recommended to exclude a CPA lesion (primarily vestibular schwannoma).
Treatment TREAT AS SOON AS POSSIBLE! Patients should be treated with a short course of oral high dose steroids (prednisone) if no contraindications exist. Relative contraindications to systemic steroid use include Cushing s syndrome, diverticulitis, peptic ulcer disease and bleeding ulcers, diabetes, heart failure, osteoporosis, psychosis, renal disease, and ulcerative colitis. It is important to start active treatment as soon as possible. The acute phase is within three weeks of the initial symptoms. After three weeks the opportunity to treat SSHL is greatly reduced.
Dosage Call local hospital ORL registrar to confirm dosage, however current accepted regimen is:
Management of SSHL Step 4: Essential to arrange repeat audiometry to determine whether hearing has recovered following the completion of medication. If there is no improvement the ORL Specialist may consider intratympanic steroid infiltration for salvage therapy e.g. dexamethasone - 3 doses 7 days apart.
Prognosis Around 50-65% of patients will recover some or all of their hearing, usually within the first two weeks after onset. Negative prognostic factors include: Age younger than 15 years or older than 60 years Vertigo Bilateral SSHL Severe to profound hearing loss Delayed onset of treatment Hearing improvement may reflect spontaneous recovery rather than a true response to medical treatment.
Hearing recovery Patient presented with SSHL and tinnitus in his left ear following acoustic trauma. No improvement despite oral and intratympanic steroid treatment. Recovery in hearing noticed around 8 months later.
Management of SSHL Step 5: Possible outcomes: recovery of normal hearing; partial recovery with residual hearing loss; no improvement. If hearing does not improve, or does not completely return to normal, a consultation with an audiologist to discuss rehabilitation options is recommended.
Rehabilitation- partial loss This patient has partial hearing loss in both ears, much worse in the left ear where the overlay of sudden loss has occurred. This patient would benefit from a pair of conventional hearing aids, given the excellent speech discrimination at elevated presentation levels.
Single-sided deafness (SSD) Defined as a severeto-profound hearing loss in one ear and normal or nearnormal hearing in the other ear. The key factor is that one ear has no usable hearing and cannot benefit from amplification. Dilworth Hearing 2018
Is one ear enough? Single-sided deafness imposes a substantial degree of burden and can lead to negative effects on psychological well-being and restrictions on social participation. A hearing loss in to one ear cannot therefore be assumed to have only minimal effects on well-being.
Functional consequences of SSD Sound localisation Difficulty identifying which direction a sound is coming from I didn t know where the traffic was, it just seemed to be all around and that was quite scary. (Lucas et al., 2018) http://sites.music.columbia.edu/cmc/musicandcomputers/chapter5/05_03.php Poor awareness of sounds/speech on the poorer hearing side The head shadow effect https://www.audiologyonline.com/articles/understanding-single-sided-deafness-evaluation-6829 Dilworth Hearing 2018
Functional consequences of SSD Difficulty understanding speech in the presence of background noise One of the major difficulties reported by individuals with SSD Following conversations in noisy environments will be challenging as it becomes very difficult to separate speech from background noise. If everyone s talking at once, it s very difficult to extract one person s conversation. It s just a noise. (Lucas et al., 2018) Speech perception abilities are also affected by the acoustics of a setting. Dilworth Hearing 2018
Functional consequences of SSD Increased listening effort and fatigue Listening with one ear, particularly in background noise, is associated with high levels of fatigue. A recent study by Alhanbali et al. (2017) compared bilateral hearing aid wearers, cochlear implant users, individuals with SSD, and normal hearing controls. All hearing-impaired groups reported significantly increased effort and fatigue compared to the control group. There was no significant difference in mean effort or fatigue between the three groups of hearing-impaired adults. Dilworth Hearing 2018
Psychological consequences of SSD People with SSD report that their hearing loss has a significant effect on both their mental and emotional wellbeing. Sudden losses are often associated with shock, fear, and devastation, and in some cases feelings of anxiety and depression. A prominent and recurring concern reported by those with SSD is anxiety regarding losing the hearing in their good ear. Many individuals report that they feel there is a social stigma about their hearing loss and comment that they often feeling stupid or embarrassed due to their communication difficulties Some felt like a hindrance as they had to rely on other people to be involved in a conversation, or felt guilty if they had missed what someone had said to them. From Lucas et al. (2018) Dilworth Hearing 2018
Social consequences of SSD Those with SSD also report that it has a significant effect on their social interactions, particularly with unfamiliar people. Social events where background noise is present is particularly hard for people with SSD and many report feeling marginalised at social events. Evidence suggests that this can lead those with SSD to withdraw from situations or withdraw within social situations where they are struggling to participate in conversations. Many workplaces also present challenging listening environments and SSD can impact social interactions in the workplace and perceived job performance. From Lucas et al. (2018) Dilworth Hearing 2018
Rehabilitation options Communication strategies CROS/BICROS BAHA Cochlear implant
Rehabilitation- dead ear This patient has total loss (dead ear) in their left ear, accompanied by no useful hearing for speech. If this loss remains after treatment, they would benefit from a CROS hearing aid system, which transfers sound from the dead side to the good ear.
Phonak CROS/BiCROS Follow conversations in quiet and noisy surroundings without having to reposition yourself Stay focused and understand speech even in noisy environments Engage in conversations that are happening on the side of your non-hearing ear No surgical procedures required
Patient L Sudden onset vertigo approx 2.5 hrs Vomiting Hearing drop and increased tinnitus on R No prednisone prescribed Changed to BiCROS system
Bone Anchored Hearing Aid (BAHA) A sound processor is clipped onto an abutment surgically implanted being the deaf ear. Sound from the poorer hearing side is sent via direct bone-conduction to the better hearing ear. Similar (or better) outcomes than CROS/BiCROS without the need to wear two devices, but requires surgery.
Cochlear implants Cochlear implants bypass the acoustic functioning of the ear completely, instead relying on electrodes to stimulate the nerves directly. Restoration of the binaural signal Requires the brain to integrate electric and acoustic stimuli Improvement to spatial orientation, speech perception in noise and tinnitus suppression.
Summary
Questions? Any further questions can be directed to: Melissa.b@dilworth.co.nz