Hearing Loss and Risk Factors in Very Low Birth Weight Infants.

The incidence of sensorineural hearing loss (SNHL) is still high in very low birth weight (VLBW) infants. The purpose of our study was to provide the prevalence rates of SNHL and to analyze the risk factors of hearing impairment and changes in hearing thresholds in a cohort of VLBW infants. A retrospective observational study was conducted in our neonatal intensive care unit (NICU) from 2012 to 2016. All VLBW infants included were screened by transient evoked otoacoustic emissions (TEOAEs) and diagnostic auditory brainstem response (ABR). In total, we enrolled 316 infants and SNHL was diagnosed in 68, leading to an early incidence of 21.5% as 36 infants out of 68 improved. Finally, SNHL was confirmed in 20 patients (6.3%) who needed hearing aids. They were significantly smaller, sicker, had longer hospitalizations, and received more ototoxic therapies. Logistic regression analysis showed that gestational age (GA) influenced the association between drugs and SNHL. The results underlined how the total exposure to antibiotics is significantly associated with SNHL, even after GA correction. In conclusion, GA, birth weight and, above all, the length and complexity of NICU stay quantify the risk of SNHL and should be considered at the individual level for parent counseling.

Acute and post-acute multidisciplinary outcomes of newborns born from mothers with SARS-CoV-2 infection during pregnancy or the perinatal period.

INTRODUCTION: We performed a single-center, prospective, observational study of newborns born from mothers with microbiologically confirmed SARS-CoV-2 infection in pregnancy or at time of delivery to evaluate acute and mid-term multidisciplinary outcomes. METHODS: Infants were offered a multidisciplinary follow-up consisting of nasopharyngeal Polymerase Chain Reaction test at birth and at 48-72 h of life, auxological and ophthalmological assessments, and serologic testing. RESULTS: 791 women and their 791 children (52.3% males) were included. Most placentas (94.9%) had abnormal inflammatory findings. 171 (27.3%) and 36 (13.7%) children respectively had pathological TEOAEs in at least one ear and bilaterally, while only four of the 85 children that underwent ABR had pathological findings (4.7%). 64 children underwent fluorescein angiography, which resulted pathological only in 1 case (1.6%). Anti-SARS-CoV-2 IgGs were found in up to 60% of children tested at six months of age. Our findings showed no association between the maternal vaccination status or the presence of maternal symptoms during pregnancy and neonatal outcomes. CONCLUSIONS: Our study shows that the large majority of newborns exposed to SARS-CoV-2 infection in utero or during the first hours of life have optimal outcomes. Our previous report of abnormal ophthalmologic findings was not confirmed on a larger cohort, while further studies are needed to better characterize audiological outcomes. Further prospective, case-controlled studies are still needed.

Hearing Impairment and Neuroimaging Results in Mitochondrial Diseases.

Mitochondrial diseases (MDs) are heterogeneous genetic disorders characterized by mitochondrial DNA (mtDNA) defects, involving tissues highly dependent on oxidative metabolism: the inner ear, brain, eye, skeletal muscle, and heart. We describe adult patients with genetically defined MDs, characterizing hearing function and neuroimaging results. We enrolled 34 patients (mean age: 50.02 ± 15 years, range: 18-75 years; 20 females and 14 males) classified in four groups: MELAS, MIDD, PEO, and Encephalopathy/Polyneuropathy. Audiological evaluations included psychoacoustical tests (pure-tone and speech audiometry), electrophysiological tests (Auditory Brainstem Responses, ABRs), and Impedenzometry. Neuroimaging evaluations considered global MRI abnormalities or structural brain changes. In total, 19/34 patients carried the m.3243A > G mutation (6 affected by MELAS, 12 affected by MIDD, and 1 affected by PEO); 11 had an mtDNA deletion (all affected by PEO); 3 had nuclear genes associated with MDs (POLG1 and OPA1); and 1 patient had an mtDNA deletion without an identified nuclear gene defect (affected by PEO). Sensory neural, bilateral, and symmetrical hearing loss was present in 25 patients (73.5%) to different degrees: 9 mild, 9 moderate, 5 severe, and 2 profound. The severe/profound and mild hearing losses were associated with pantonal and high-frequency audiograms, respectively. Instead, moderate hearing losses were associated with both high-frequency (five cases) and pantonal (five cases) audiogram shapes. In addition, 21/25 patients showed a cochlear site of lesion (84%), and 4/25 (16%) showed a retrocochlear site. We found global MRI abnormalities or structural brain changes in 26/30 subjects (86.6%): 21 had white matter abnormalities, 15 had cortical atrophy, 10 had subcortical atrophy, 8 had basal nuclei involvement or cerebellar atrophy, 4 had stroke-like lesions or laminar necrosis, and 1 had cysts or vacuolated lesions. We concluded that genetic alterations are associated with different clinical presentations for both auditory function and neuroradiological findings. There is no fixed relationship between genotype and phenotype for the clinical conditions analyzed.

Clinical Evolution of Acute Vestibular Syndrome: Longitudinal Retrospective Analysis of Epidemiological Data and Prognostic Factors for Recovery.

We evaluated the clinical features of patients admitted to the ED with acute vertigo, verifying symptoms after one month and one year to establish epidemiological factors and predictors of resolution. We reviewed 233 records of patients admitted to ED for acute vertigo (125 F and 85 M, mean age 56.12 years). We analyzed the correlation between time of resolution (at one month and one year) and symptoms duration (subjective and/or objective vertigo, instability, cervical pain, audiological, neurological, and neurovegetative symptoms), comorbidities, and therapies, the result of the clinical and instrumental vestibular examination. Resolution of acute vertigo occurred in 81.1%, while persistence of vertigo one year after ED access was reported in 18.8%. There were 135 patients who recovered in one month. The presence of instability, auditory and neurovegetative symptoms, and neck pain represents a significant factor for recovery within one year. Age over 65 and a history of hypertension are associated with a worst recovery. Patients with spontaneous Nystagmus or positive HIT showed a significant difference in symptoms recovery within one month and one year. Presence of positional Nystagmus represents a positive prognostic factor. Our findings emphasize the importance of clinical evaluation of the acute vertigo, helping the clinicians to define central or peripheral diagnosis and predict the resolution of vertigo.

Posturographic Analysis in Patients Affected by Central and Peripheral Visual Impairment.

Although vision loss is known to affect equilibrium maintenance, postural control in patients affected by low vision has been poorly investigated. We evaluated postural stability and the ability to use visual, proprioceptive and vestibular information in different low vision patterns. Ten adults with normal vision (NC), fourteen adults affected by central visual impairment (CLV) and eight adults affected by peripheral visual impairment (PLV) were enrolled in our study. Patients underwent visual, vestibular and postural evaluation (bedside examination, Computed Dynamic Posturograophy). Motor Control Tests were performed to analyze automatic postural adaptive responses elicited by unexpected postural disturbances. Clinical evaluations did not show abnormality in all patients. In the Sensory Organization Test, CLV and PLV patients performed more poorly in conditions 3-6 and 3-4, as compared to NC subjects. The condition 5 score was significantly lower in the CLV group with respect to the PLV patients. Composite equilibrium scores demonstrated significant differences between low-vision subjects vs. NC subjects. No differences were found for somatosensorial contribution. Visual afferences showed lower values in all visually impaired subjects, while vestibular contribution was lower in the CLV patients as compared to the NC and PLV patients. MCT latencies were significantly worse in the CLV subjects. In the low-vision patients, postural control was modified with a specific pattern of strategy adaptation. Different modulations of postural control and different adaptive responses seemed to characterize CLV patients as compared to PLV subjects.

Spyramicine and Trimethoprim-Sulfamethoxazole Combination to Prevent Mother-To-Fetus Transmission of Toxoplasma gondii Infection in Pregnant Women: A 28-Years Single-center Experience.

BACKGROUND: There is weak evidence on the best treatment of pregnant women with Toxoplasma gondii infection to prevent the vertical transmission to the fetus. METHODS: We conducted a 28-year retrospective study aiming to compare the efficacy of three therapeutic regimens [Spiramicyn alone (Spy) vs. Pyrimethamine-Sulfadiazine (P/S) vs. Spiramicyn with Trimethoprim-Sulfamethoxazole (Spy+TMP-SMX)] for the prevention of mother-to-fetus transmission of T. gondii infection. RESULTS: 170 women were included: 58 (34.1%) had certain congenital toxoplasmosis (CT), 61 (35.9%) a probable infection and 41 (24.1%) possible infection. In total 97 mothers (57.1%) were treated with the Spy+TMP-SMX combination, 64 mothers (37.6%) were treated with Spy only and 8 mothers (4.7%) with P/S. Infected infants were 20/170 (11.7%). However, 8.2% (8/97) of infants born to mothers treated with Spy+TMP-SMX were infected, 20% (11/55) of infants born to women treated with Spy and 12.5% (1/8) of infants born to mothers treated with P/S were infected. Logistic regression analysis demonstrated that Spy treatment alone was associated with an increased risk of CT compared to the Spy+TMP-SMX combination (OR, 2.78, 95% CI 1.04-7.41, P value 0.041). No difference was observed when the Spy+TMP-SMX was compared with the P/S combination (OR 1.59; 95% CI 0.17 - 14.58; P value 0.682). Results were confirmed when the analyses were corrected by trimester of infection and by type of maternal treatment (OR 7.72; 95% CI 3.40-17.53, P value <0.001). CONCLUSIONS: The combination of Spy+TMP-SMX may be more effective in reducing the risk of maternal-fetal transmission of Toxoplasmosis compared to Spy alone; furthermore, this combination is not inferior to P/S, the current international standard-of-care maternal treatment for the prevention of CT. A prospective trial comparing the combination Spy+TMP-SMX with P/S would be necessary to provide definitive evidence on the best regimen for pregnant women with T. gondii infection.

Short- and mid-term multidisciplinary outcomes of newborns exposed to SARS-CoV-2 in utero or during the perinatal period: preliminary findings.

The long-term outcomes of newborns exposed to SARS-CoV-2 infection in utero or during the first hours of life are still unknown. We performed a single-center, prospective, observational study of newborns born from mothers with microbiologically confirmed SARS-CoV-2 infection in pregnancy or at time of delivery. Infants were offered a multidisciplinary follow-up consisting of nasopharyngeal Polymerase Chain Reaction test at birth and at 48-72 h of life, auxological growth and neurological development, serologic testing, and audiological and ophthalmological assessments. One-hundred ninety-eight mothers and 199 newborns were enrolled. Of the 199 newborns, 171 underwent nasopharyngeal swab, four (2.3%) and two (1.15%) children tested positive at birth and 48-72 h of life, respectively. None had SARS-CoV-2 related symptoms. Auxologic and neurologic development were normal in all children during follow-up. Nine out of 59 infants had SARS-CoV-2 IgG at 3 months of life, which was associated with a positive nasopharyngeal swab at birth (P = 0.04). Twenty seven out of 143 (18.8%) newborns had pathologic transitory evoked otoacoustic emissions at birth, although 14/27 repeated after 1 month were normal. Audiological evaluation was completed with Auditory Brainstem Response between the third and sixth month of life in 34 children, showing in all normal hearing threshold. The ophthalmological evaluation found retinal vascular anomalies in 3/20 (15%) children, immature visual acuity in 5/20 (25%) children, and reduced distance attention in 6/20 cases (30%). CONCLUSIONS: Our study showed that the neonatal and mid-term multidisciplinary outcomes of newborns exposed to SARS-CoV-2 infection in utero or during the first hours of life are mostly positive, with the exception of ophthalmologic findings which, in a preliminary cohort, were abnormal in about 15% of cases. Further prospective, longitudinal studies are needed to better understand the clinical outcomes of children exposed to SARS-CoV-2 in utero and in the early postnatal life. WHAT IS KNOWN: • In utero mother-to-child transmission of SARS-CoV-2 has been documented by several independent studies. • Neonatal COVID-19 is a systemic disease that can be severe, although rarely. WHAT IS NEW: • Newborns exposed in utero to SARS-CoV-2 have mostly a normal auxological, audiological, and neurological development during the first months of life. • Fundus fluorescein angiography revealed that up to 5% of newborns exposed in utero to SARS-CoV2 can show retinal and choroidal abnormalities, including peripheral hypofluorescence of the choroid and increased vascular tortuosity.

Embryopathy Following Maternal Biliopancreatic Diversion: Is Bariatric Surgery Really Safe?

Pregnancy after bariatric surgery is usually considered safe. Recently, a few studies reported that bariatric surgery represents a risk factor for birth defects. A case series of six patients, born from women who had undergone biliopancreatic diversion, is reported. The clinical pattern was characterized by psychomotor development delay (100%), microphthalmia (83%), growth retardation (66%), hearing loss (66%), and variable facial dysmorphism. Based on the clinical profile and symptoms reported by women during pregnancy, a causal association between maternal chronic post-surgical malabsorption, congenital anomalies, and neonatal outcome is proposed, with vitamin A deficiency representing a major causing factor. Educational follow-up support, continuous clinical monitoring, and appropriate nutritional assessment appear to be crucial to reduce the potential risk of congenital malformations and child disability.

Long-Term Clinical, Audiological, Visual, Neurocognitive and Behavioral Outcome in Children With Symptomatic and Asymptomatic Congenital Cytomegalovirus Infection Treated With Valganciclovir.

Cytomegalovirus (CMV) is the most common cause of congenital infection in humans. There are no enough data on long-term outcome of newborns with congenital CMV (cCMV) infection, particularly for those asymptomatic at birth. For this reason, we performed this study to evaluate long-term audiological, visual, neurocognitive, and behavioral outcome in patients with symptomatic and asymptomatic cCMV infection treated with oral Valganciclovir (VGC). Thirty-six newborns with confirmed cCMV infection were evaluated: 12 (33.3%) symptomatic at birth and 24 asymptomatic (66.7%). No one had cognitive impairment. Cognitive assessment scales resulted abnormal in 4/35 patients (11.4%). 11/21 patients (52.4%) achieved abnormal scores in neuropsychological tests. The language evaluation gave pathological results in 6/21 (28.5%) patients. 6/35 patients (17.1%) developed SNHL, all symptomatic at birth except one. None of the 34 patients evaluated developed CMV retinopathy. Our study shows that both symptomatic and asymptomatic newborns with cCMV infection develop long-term sequelae, particularly in the behavioral and communicative areas, independently from the trimester of maternal infection.

Does the involvement of first-year residents have a negative impact on the performance of a newborn hearing screening program?

OBJECTIVES: We aimed to evaluate the efficiency of our hearing screening program, prior to hospital discharge, together with the consistency of our teamwork including first year residents by assessing a learning curve for the operators involved. METHODS: We evaluated all the data collected during the first stage of the screening program of all non-NICU neonates from March 2009 to July 2013, analyzing by means of a linear regression model, the monthly referral rate for the whole period of activity of each group of residents. RESULTS: performances of each group of screeners were statistically different (chi square test p < 0.005). The nptrend test showed that group 2 (p = 0.01) and group 4 (p = 0.01) reached a statistical significance in higher and lower referral rates respectively. No statistical differences were found in other groups (Group 1 p = 0.161; Group 3 p = 0.853). CONCLUSION: Despite a statistically significant difference in the performances between the groups of residents, the referral rates for each group (range 6.18%-9.29%) and the overall referral rate for the whole period (7.84%) agree with the values commonly reported for TEOAEs in the literature. It means that our screening program is reasonably effective despite a yearly turnover of operators.

Does Hearing Loss in the Elderly Individuals Conform to Impairment of Specific Cognitive Domains?

BACKGROUND: It is well known that age-related hearing loss (ARHL) is strongly associated with dementia. Different hypotheses have been considered to explain this link, including sensorial deprivation, the reduction in cognitive reserve, and the presence of shared pathological pathways (microvascular damage of the brain). AIMS: We carried out a study of the audiological and neuropsychological characteristics of a sample of hearing impaired but cognitively healthy individuals (HIH). The aim of our study was to carefully outline the neuropsychological profile of the patients in order to verify whether hearing loss correlated with deficits in specific cognitive domains. RESULTS: Episodic memory is affected by the presence of hearing loss, while semantic competences, syntactic, and grammar skills seem not to be affected. Furthermore, some audiological features linked to the intelligibility of spoken words can predict the presence of executive dysfunction; the same does not apply to memory impairment. CONCLUSION: In HIH, executive functions are widely employed in maintaining an acceptable level of comprehension of spoken language; consequently, other cognitive domains and instrumental abilities in HIH are not properly supported. Thus, it is arguable that programs of hearing rehabilitation for HIH could restore the allocation of attentional resources to the functioning of other cognitive domains.

Prospective Evaluation of Cognitive Functions After Rehabilitation With Cochlear Implant or Hearing Aids: Preliminary Results of a Multicentric Study on Elderly Patients.

Objective Recent literature has shown a growing interest in the relationship between presbycusis and cognitive decline, but significant evidence about the long-term benefit of rehabilitation on cognitive functions has not been reported yet. The aim of the study was to analyze audiological and neuropsychological performances in patients with cochlear implant (CI) or hearing aids (HAs) over time. Materials and Method Forty-four bilaterally deaf patients aged more than 60 years (25 with CI candidacy and 19 with HA candidacy) were enrolled. Patients were subjected to audiological evaluation, to a battery of neuropsychological tests (Mini-Mental State Examination [MMSE], Rey Auditory Verbal Learning Task [RAVLT], Rey-Osterreith Complex Figure Test, Digit/Corsi Span Forward and Backward, Multiple Features Target Cancellation, Trail-Making Test, Stroop Test, and Phonological and Semantic Word Fluency), and to a quality of life assessment (Short Form 36, Glasgow Benefit Inventory, Glasgow Health Status Inventory) at the baseline and after a long-term follow-up (6-12 months). Results Speech recognition scores in quiet and in noise were significantly improved even 6 months after auditory rehabilitation. Significant differences between pre- and post-rehabilitation scores were reported in physical and emotional impacts in life, general global health, vitality, and social activities. MMSE and RAVLT scores were significantly improved in both groups after 6 months of follow-up, suggesting a global involvement of memory domain. Mnesic performances remained unchanged between the first and second follow-up, but a further significant improvement in executive functions (Stroop Test) was detected in patients with CI reevaluated 12 months after implantation. A significant correlation of the RAVLT with signal-to-noise ratio at +10 dB speech-in-noise scores and the MMSE with signal-to-noise ratio at 0 dB speech-in-noise scores suggests the pivotal role of executive functions in recognition in noisy environment. Conclusions Our preliminary data confirm that hearing deprivation in aged patients represents a truly modifiable risk factor for cognitive decline, which can be positively faced by acoustic rehabilitation. The improvement of short- and long-term memory performances and the amelioration of executive and attentive functions suggest that hearing restoration with both HAs and CI may provide a recovery of superior cognitive domains probably through a reallocation of cortical resources altered by hearing deprivation.

Universal newborn hearing screening in the Lazio region, Italy.

BACKGROUND: The introduction of Universal Newborn Hearing Screening (UNHS) programs has drastically contributed to the early diagnosis of hearing loss in children, allowing prompt intervention with significant results on speech and language development in affected children. UNHS in the Lazio region has been initially deliberated in 2012; however, the program has been performed on a universal basis only from 2015. The aim of this retrospective study is to present and discuss the preliminary results of the UNHS program in the Lazio region for the year 2016, highlighting the strengths and weaknesses of the program. METHODS: Data from screening facilities in the Lazio region for year 2016 were retrospectively analyzed. Data for Level I centers were supplied by the Lazio regional offices; data for Level II and III centers were provided by units that participated to the study. RESULTS: During 2016, a total of 44,805 babies were born in the Lazio region. First stage screening was performed on 41,821 children in 37 different birth centers, with a coverage rate of 93.3%. Of these, 38.977 (93.2%) obtained a "pass" response; children with a "refer" result in at least one ear were 2844 (6.8%). Data from Level II facilities are incomplete due to missing reporting, one of the key issues in Lazio UNHS. Third stage evaluation was performed on 365 children in the three level III centers of the region, allowing identification of 70 children with unilateral (40%) or bilateral (60%) hearing loss, with a prevalence of 1.6/1000. CONCLUSIONS: The analysis of 2016 UNHS in the Lazio region allowed identification of several strengths and weaknesses of the initial phase of the program. The strengths include a correct spread and monitoring of UNHS among Level I facilities, with an adequate coverage rate, and the proper execution of audiological monitoring and diagnosis among Level III facilities. Weakness, instead, mainly consisted in lack of an efficient and automated central process for collecting, monitoring and reporting of data and information.

Audiologic Assessment in Adults With Down Syndrome.

Increased life expectancy in persons with Down syndrome (DS) is associated with premature age-related changes. The aim of this study was to assess auditory function in adults with DS and to evaluate the prevalence of hearing loss in this population. Audiometric tests were performed in 72 adults with DS (mean age 37.3±10.1 years, 51.4% females). Air conduction pure tone average (PTA) thresholds at frequencies 0.5-1-2-4 kHz were calculated to assess hearing function. Hearing loss was present if the PTA threshold was > 20 dB hearing level. Higher frequencies of 4 and 8 kHz were also assessed. Hearing loss was shown in 47 (65.3%) participants. The prevalence of hearing loss increased with age, ranging from 42.86% in the 20-29 years group to 90.91% in the 50-59 years group. High frequencies (4 and 8 kHz) were more often impaired than other frequencies used to measure PTA. Thus, the study concluded hearing loss is common in adults with DS and shows a pattern compatible with precocious aging of the hearing system. Auditory evaluation is strongly recommended in adults with DS.

Relationship between Subjective Tinnitus Perception and Psychiatric Discomfort.

INTRODUCTION: Tinnitus patients have higher risk of developing anxiety-depressive disorders and decreased quality of life. The reasons why selected patients are able to cope with chronic tinnitus, whereas it represents a disabling symptom for others remain under discussion. OBJECTIVES: the objective of the study was to determine the tinnitus-related degree of distress along with the prevalence of anxiety-depression disorders in a sample of eighty patients referring for chronic tinnitus at the Department of Otolaryngology of Catholic University of Rome from March to September 2015. MATERIALS AND METHODS: We administered to all patients the Italian versions of Tinnitus Handicap Inventory (THI) and Hospital Anxiety and Depression Scale (HADS). Furthermore we investigated the correlation among patient's discomfort, severity of hearing loss and age. RESULTS: Average THI score was 40.85, meaning moderate degree of discomfort; 57.5% of the patients showed HADS scores consistent with high risk of psychiatric comorbidities. A significant linear correlation between THI and HADS scores was demonstrated. CONCLUSION: We suggest that patients with severe tinnitus-related distress are routinely invited to accomplish psychometric questionnaires, to assess the possibility of a neuropsychiatric evaluation and/or specific pharmacological planning. At this purpose we recommend the administration of HADS, as a reliable and quick instrument.

Steady state auditory evoked potentials in normal hearing subjects: evaluation of threshold and testing time.

BACKGROUND: Steady state responses (SSRs), between 75 and 110 Hz, evoked by auditory amplitude modulated single or multiple tone stimuli, may be used to estimate objective hearing threshold. METHODS: The aim of this study was to compare SSRs and click-evoked auditory brainstem responses (ABRs) in both ears of 20 adults (10 males and 10 females, aged between 24 and 36 years) with normal hearing threshold. RESULTS: Mean ABR threshold was found at 21.25 (± 5.9) dB nHL. Mean SSR threshold was found at 15.6 (± 9.6) dB nHL after a single frequency stimulus (1 kHz); at 10.5 (± 18.2) dB nHL and at 7.1 (± 12.4) dB nHL after bifrequency stimulation (0.5 and 2 kHz). SSR thresholds after multifrequency stimulation (0.5, 1, 2 and 4 kHz) were found, respectively, at 12.1 (± 12.9) dB nHL, 12.2 (± 12.8) dB nHL, 12.3 (± 8.3) dB nHL and 18.9 (± 17.2) dB nHL. Mean duration of the recording session was 6 min in the case of ABRs, while it was 25 min in the single frequency condition and 29 min in the multifrequency condition in the case of SSRs. CONCLUSION: SSRs can be used for frequency-specific objective audiometry. The multifrequency stimulation greatly reduces the whole testing time.

Auditory processing in infancy: do early abnormalities predict disorders of language and cognitive development?

Increasing attention has been devoted to the maturation of sensory processing in the first year of life. While the development of cortical visual function has been thoroughly studied, much less information is available on auditory processing and its early disorders. The aim of this paper is to provide an overview of the assessment techniques for early auditory processing. While otoacoustic emissions and auditory brainstem responses are well-established tools for neonatal screening of hearing loss, there have been less consistent results for neurophysiological assessments of central auditory processing in clinical practice. Early auditory event-related potentials could provide valuable diagnostic information, but their use as a possible clinical screening method is still limited and should be further assessed. Behavioural tests are few and are greatly needed in young infants as they could provide a more easily used tool for detecting the preconditions of early cerebral auditory impairment.

Sudden onset sensorineural hearing loss caused by meningeal carcinomatosis secondary to occult malignancy: report of two cases.

Meningeal carcinomatosis (MC) is an uncommon form of metastasis of solid tumors. In the absence of clinical meningeal or parenchymal involvements, the sensorineural hearing loss (SNHL) as the starting symptom of MC is very infrequent. We report the history of two patients affected by MC who presented first with progressive SNHL. In both cases the early magnetic resonance imaging (MRI) finding mimicked bilateral masses in the cerebellopontine angle (CPA). Only the histopathologic result and surgical biopsy in cases 1 and 2, respectively, identified masses secondary to occult malignancy. However the available investigations could not discover the primary site of metastatic carcinoma. Despite the poor prognosis, because of the rarity and severity of MC we consider important to make known our experience in order to consider metastatic tumors in the differential diagnosis for sudden SNHL.