Somatic modulation of tinnitus: a review and some open questions

Tinnitus modulation by movements of the temporomandibular joint, head and neck musculoskeletal structures and the eye, can be found in one to two thirds of tinnitus sufferers; unfortunately this condition is often overlooked by otolaryngologists. Although somatic modulation has been initially hypothesized as a fundamental characteristic of tinnitus, there is increasing evidence of a tight connection with disorders of non-auditory regions. The structure that mostly modulates tinnitus is the temporomandibular joint, which mainly causes an increase in tinnitus loudness, followed by head and neck movements that may result in an increase or decrease of loudness and eye movements (gaze-evoked tinnitus). Besides loudness, somatic movements can also modulate tinnitus pitch and localization. Somatosensory tinnitus is a relatively new nding that leaves several open questions: are there individual predisposing factors to somatic modulation? How strong is the association between the capability to somatically modulate tinnitus and an underlying non-auditory disorder? Changes that occur after somatic maneuvers are only transitory? Why patients that have concomitant hyperacusis also have higher chances of tinnitus modulation? Further basic science and clinical research is required to address these and many other questions about somatosensory tinnitus

Metastatic renal cell carcinoma presenting as a paranasal sinus mass: the importance of differential diagnosis

Metastases in the paranasal sinuses are rare; renal cell carcinoma is the most common cancer that metastasizes to this region. We present the case of a patient with a 4-month history of a rapidly growing mass of the nasal pyramid following a nasal trauma, associated with spontaneous epistaxis and multiple episodes of hematuria. Cranial CT scan and MRI showed an ethmoid mass extending to the choanal region, the right orbit, and the right frontal sinus with an initial intracranial extension. Patient underwent surgery with a trans-sinusal frontal approach using a bicoronal incision combined with an anterior midfacial degloving; histological exam was compatible with a metastasis of clear cell renal cell carcinoma. Following histological findings, a total body CT scan showed a solitary 6 cm mass in the upper posterior pole of the left kidney identified as the primary tumor. Although rare, metastatic renal cell carcinoma should always be suspected in patients with nasal or paranasal masses, especially if associated with symptoms suggestive of a systemic involvement such as hematuria. A correct early-stage diagnosis of metastatic RCC can considerably improve survival rate in these patients; preoperative differential diagnosis with contrast-enhanced imaging is fundamental for the correct treatment and follow-up strategy

Somatosensory tinnitus: current evidence and future perspectives

In some individuals, tinnitus can be modulated by specific maneuvers of the temporomandibular joint, head and neck, eyes, and limbs. Neuroplasticity seems to play a central role in this capacity for modulation, suggesting that abnormal interactions between the sensory modalities, sensorimotor systems, and neurocognitive and neuroemotional networks may contribute to the development of somatosensory tinnitus. Current evidence supports a link between somatic disorders and higher modulation of tinnitus, especially in patients with a normal hearing threshold. Patients with tinnitus who have somatic disorders seems to have a higher chance of modulating their tinnitus with somatic maneuvers; consistent improvements in tinnitus symptoms have been observed in patients with temporomandibular joint disease following targeted therapy for temporomandibular disorders. Somatosensory tinnitus is often overlooked by otolaryngologists and not fully investigated during the diagnostic process. Somatic disorders, when identified and treated, can be a valid therapeutic target for tinnitus; however, somatic screening of subjects for somatosensory tinnitus is imperative for correct selection of patients who would benefit from a multidisciplinary somatic approach

Relationship between hearing function and myasthenia gravis: a contemporary review

There is increasing evidence of a connection between hearing function and myasthenia gravis (MG). Studies of the pathophysiological basis of this relationship suggest that acetylcholine receptors (AChRs) on outer hair cells (OHCs) play a central role. In patients with MG, autoantibodies against AChRs induce a progressive loss of AChRs on OHCs, decreasing their electromotility. The stapedial reflex decay test can be altered in MG patients, and can be used as an additional tool for diagnosis and monitoring. Transient evoked and distortion product otoacoustic emissions are the main diagnostic tool for monitoring OHC functionality in MG patients, and can be used to record subclinical hearing alterations before the onset of clinically evident hearing loss. Understanding the association between MG and hearing dysfunction requires a multidisciplinary approach. Otolaryngologists should take this relationship into account when approaching patients with a diagnosis of myasthenia gravis and "in patients with MG" with ण128;\u9cin MG patients, and the progress of hearing alterations should always be monitored in patients with MG

Adult-type rhabdomyoma of the larynx: clinicopathologic study of an uncommon tumor in a rare location

Rhabdomyoma is an uncommon benign mesenchymal tumor with skeletal muscle differentiation that may occur either in the heart or in extra-cardiac sites. Even though the head and neck region is the most common area of extra-cardiac rhabdomyoma, the larynx is rarely involved. We present the case of an 85-year-old woman who reported a 10-day history of breathing difficulties, dysphagia and dysphonia. A computed tomography scan of the head and neck showed a contrast-enhanced, solid hypopharyngeal-laryngeal neoplasm with well-defined margins causing subtotal obliteration of the right pyriform sinus and a reduction in air lumen of the laryngeal vestibule. The patient underwent complete endoscopic removal of the lesion; histologic examination revealed an adult- type rhabdomyoma based on the histologic features and the immunoreactivity of the neoplastic cells for desmin, myoglobin and muscle-specific actin but not for cytokeratin, S-100, CD68R, chromogranin-A and synaptophysin. Since clinical and imaging features are not specific for rhabdomyoma, histologic examination and immunohistochemical analyses play a central role in the differential diagnosis of the adult-type rhabdomyoma from other laryngeal neoplasms. A correct diagnosis is mandatory to avoid inappropriate treatment

Vestibular schwannoma and ipsilateral endolymphatic hydrops: an unusual association

Vestibular schwannoma and endolymphatic hydrops are two conditions that may present with similar audio-vestibular symptoms. The association of the two in the same patient represents an unusual nding that may lead clinicians to errors and delays in diagnosis and clinical management of affected subjects. We discuss the case of a patient with an intrameatal vestibular schwannoma reporting symptoms suggestive for ipsilateral endolymphatic hydrops. The patient presented with uctuating hearing loss, tinnitus, and acute rotatory vertigo episodes, and underwent a full audiological evaluation and imaging of the brain with contrast-enhanced Magnetic Resonance Imaging. Clinical audio-vestibular and radiological examination con rmed the presence of coexisting vestibular schwannoma and endolymphatic hydrops. Hydrops was treated pharmacologically; vestibular schwannoma was monitored over time with a wait and scan protocol through conventional MRI. The association of vestibular schwannoma and endolymphatic hydrops is rare, but represents a possible nding in clinical practice. It is therefore recommended investigating the presence of inner ear disorders in patients with vestibular schwannoma and, similarly, to exclude the presence of this condition in patients with symptoms typical of inner ear disorders

Recovery from repeated sudden hearing loss in a patient with Takayasu’s arteritis treated with hyperbaric oxygen therapy: the first report in the literature

Hearing loss has been rarely reported in Takayasu's arteritis, presents as sudden sensorineural hearing loss and usually responds well to corticosteroid therapy. Hyperbaric Oxygen Therapy is commonly used as a supplementary treatment to corticosteroids for sudden hearing loss. We present the case of a woman with Takayasu's arteritis who had two episodes of hearing loss involving one ear at a time with a 11-month delay between each episode. During the first episode, the patient was treated with high-dose intramuscular corticosteroids with a temporary improvement of auditory threshold, that deteriorated close to previous level 14 days after cessation of therapy. In the second episode, Hyperbaric Oxygen Therapy was associated to corticosteroids, with a significant, longer term improvement in both ears, including the one that had shown to be unresponsive to previous pharmacologic therapy. Oxygen therapy could have played a role in hearing restoration in this patient, as it could have acted synergically with corticosteroids amplifying their effect

Characteristics of somatic tinnitus patients with and without hyperacusis

Objective: Determine if somatic tinnitus patients with hyperacusis have different characteristics from those without hyperacusis. Patients and methods: 172 somatic tinnitus patients with (n = 82) and without (n = 90) hyperacusis referred to the Tinnitus Unit of Sapienza University of Rome between June 2012 and June 2016 were compared for demographic characteristics, tinnitus features, self-administered questionnaire scores, nature of somatic modulation and history. Results: Compared to those without hyperacusis, patients with somatic tinnitus and hyperacusis: (a) were older (43.38 vs 39.12 years, p = 0.05), (b) were more likely to have bilateral tinnitus (67.08% vs 55.56%, p = 0.04), (c) had a higher prevalence of somatic modulation of tinnitus (53.65% vs 36.66%, p = 0.02) and (d) scored significantly worse on tinnitus annoyance (39.34 vs 22.81, p<0.001) and subjective hearing level (8.04 vs 1.83, p<0.001). Conclusion: Our study shows significantly higher tinnitus modulation and worse self-rating of tinnitus and hearing ability in somatic tinnitus patients with hyperacusis versus somatic tinnitus patients without hyperacusis. These differences could prove useful in developing a better understanding of the pathophysiology and establishing a course of treatment for these two groups of patients

Hyperacusis in children: a preliminary study on the effects of hypersensitivity to sound on speech and language

There is a growing awareness that children may experience hyperacusis, a condition that is often associated with behavioral and developmental disorders. This preliminary study was aimed to investigate the effects of hyperacusis alone on various components of speech and language in children without developmental disorders. This study was conducted on 109 children aged between 4 and 7 years attending kindergarten and primary school. Hyperacusis was assessed through behavioral observation of children and questionnaires for parents. Different components of speech and language were assessed through specific tests. Hyperacusis was diagnosed in fifteen children (13.8%); ten (66.7%) were attending primary school and five (33.3%) kindergarten. A significant difference between children with and without hyperacusis was found for tests evaluating the average number of words in a sentence and phonemic fluency; older children appeared to have more difficulties. Several differences in education profiles were found: parents of children with hyperacusis spent less time with their children compared to parents of children without hyperacusis. Our preliminary results suggest some difficulties in lexical access and the use of shorter sentences by children with hypersensitivity to sound; however, the small size of our sample and the largely unknown interactions between hyperacusis and developmental disorders suggest caution when interpreting these results. Further studies on larger samples are necessary to gain additional knowledge on the effects of hyperacusis on speech and language in children without developmental disorders

Subtyping patients with somatic tinnitus: modulation of tinnitus and history for somatic dysfunction help identify tinnitus patients with temporomandibular joint disorders

Objective: Determine in a cohort of patients with normal hearing and chronic tinnitus if self-reported history for temporomandibular joint (TMJ) dysfunction and a positive modulation of tinnitus in the TMJ region could be suggestive of an underlying TMJ disorder. Patients and Methods: The study included 226 patients presenting to the Head and Neck Service of our University Hospital. Following audiological and somatic tinnitus evaluation, patients were divided into two groups. The study group (n= 134) included subjects that met both the following criteria: A) a self-reported history for TMJ dysfunction and B) a positive modulation of tinnitus following somatic maneuvers in the TMJ region. The control group (n=92) included patients with similar demographic and tinnitus characteristics that did not meet the proposed criteria for somatic tinnitus. Afterwards, patients underwent clinical TMJ evaluation in the Service of Clinical Gnathology of our University. Results: One hundred thirty-one patients (57.9%) received a clinical diagnosis of TMJ disorder according to DC/TMD Axis I; 79.1% in the study group and 27.2% in the control group. Ninety-five (42.1%) patients were negative for TMJ disorders; 20.9% in the study group and 72.8% in the control group. A significantly higher number of TMJ disorders was found in patients in the study group compared to the control group (p<0.0001). Most patients had joint disorders (67.2%), followed by other (29.8%) and pain disorders (29%). Logistic regression analysis in the study group showed that female gender was more prevalent in patients with TMJ disorders. Conclusion: Our findings in patients with chronic tinnitus and normal hearing suggest that self-reported history for somatic dysfunction and modulation of tinnitus, when occurring simultaneously in the TMJ region, can be useful to preliminarily identify patients with TMJ disorders